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693 Cards in this Set
- Front
- Back
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Is musculoskeletal disease expensive?
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Estimated costs to US is 2.5% of GNP
215 billion |
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Synovia
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Clear, viscous lubricating fluid secreted by the membranes in joint cavities, sheaths, tendons, bursa
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Synovitis
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Inflammation of synovial tissue
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Bursa
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Sac-like body cavity
Especially one located between joint or at points of friction between moving structures |
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Enthesis
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Site of insertion into bone of tendon, ligament, capsule
metabolically active zone SI joints, ligamentous structures of vertebrae, manubrosternal joints, symphysis pubis, iliac crests, trochanters, patella, calcanei |
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Is this joint pain inflammatory or mechanical?
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Inflammatory: morning stiffness >1hr, fatigue, improves with activity, worsens at rest, systemic symptoms, steroid responsive
Mechanical: morning stiffness <30 min, minimal fatigue, worse with activity, no systemic illness, not steroid responsive |
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Red flags for joint pain
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Fevers, rigors
Weight loss No comfortable position Wakes from sleep Loss of function Trauma Known malignancy |
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Acute onset nontraumatic monoarticular joint pain DDx
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Gout
Pseudogout Septic arthritis |
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Acute onset nontraumatic polyarticular joint pain DDx
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RA
Parvo SLE |
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Acute onsent nontraumatic oligoarticular joint pain DDx
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Septic arthritis
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Chronic onset monoarticular joint pain DDx
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Osteoarthritis
Fungal arthritis Tumor FB |
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Chronic onset polyarticular joint pain DDx
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Osteoarthritis
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Chronic onset oligoarticular joint pain DDx
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Osteoarthritis
Psoriatric arthritis |
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Subacute onset monarticular joint pain DDx
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Lyme
AVN |
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Subacute onset polyarticular joint pain DDx
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RA
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Subactue onset oligoarticular joint pain DDx
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Reactive
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Intermittent monarticular joint pain DDx
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Gout or pseudogout
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Intermittent polyarticular joint pain DDX
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Gout
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Intermitent oligoarticular joint pain DDx
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Ankylosis spondylitis
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Osteoarthritis hands
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Firm swelling at DIPs, PIPs, first CMC
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Osteoarthritis definition
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Slowly progressive disintegration of cartilage
Secondary damage to surrounding structures |
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Primary osteoarthritis
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Very common
Pathogenesis unknown Involves hands, spine, hips, knees, first MTP |
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Secondary ostearthritis
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Osteoarthritis following an inflammatory disorder, trauma, metabolic factor
Ex excessive Fe in hemochromatosis |
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Osteoarthritis epidemiology
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Most common joint disorder
50% by age 65 80% by 75 Second to CAD in causing work disability in men >50 in US |
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Clinical presentation of OA
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Age >50
<30 minutes morning stiffness Crepitus No inflammation Bony enlargement or tenderness |
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Labs in OA
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ESR <40
RF < 1:40 Noninflammatory synovial fluid |
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Radiographic findings in RA
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Joint space narrowing
Osteophytes Subcondral cysts and sclerosis Malalignment |
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Causes of secondary OA
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Congenital/developmental
Trauma Inflammatory joint disease Endocrinopathies Metabolic diseases Neuropathic disorders Avascular necrosis Paget's disease |
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Function of hyaline cartilage in joint
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Shock absorber of the diarthroidal joints
Handles 90% of weight bearing |
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Changes to hyaline/articular cartilage in OA
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Changes in composition and molecular organization
Increased water content -- soggy More pliable -- gives more Deforms excessively with weight |
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What does degenerating cartilage look like histologically?
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Diffuse hypercellularity
Loss of acid mucopolysaccarides from matrix Then Small clefts in surface Deep vertical tears Clumping of chondrocytes |
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Late changes to joint in OA
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Progressive fibrillation and loss of cartilage
Osteophyte formation and subchondral sclerosis Modest inflammatio in synovium Ligmentous laxity Weakness of periarticular muscles Erosion of cartilage Bone on bone |
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Chondrocyte response to adverse environmental stimuli
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Promote matrix degradation
cytokines, proteinases Downregulating repair processes Make BMPs, TGFbeta -- promote osteophyte growth Not helpful for avoiding OA |
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Distribution of OA in hands of men and women
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Both have lots of DIP involvement
Some PIP involvement Majority of women (and lots of men) have thumb base involvement |
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Bouchard's nodes
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Bony enlargement of PIP
Osteoarthritis |
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Heberden's nodes
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Boney enlargement of the DIP
Osteoarthritis Can start of as synovial cyst |
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Who is likely to have hand nodes in OA?
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Women after menopause
Also genetics |
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What does an OA joint look like on xrt?
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Loss of joint space
Osteophytes Sclerosis Subcondral cysts Subluxation |
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Cause of bilateral varus knees
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Medical compartment OA
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Risk factors for OA
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Age
Gender (young men, older women) Obesity Joint injury Previous deformity Ligamentous laxity |
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What kinds of injuries increase risk of OA?
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Congenital dysplasia (hip)
Fractures through articular surface Ligament or meniscal injuries resulting in instability |
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Sports and OA
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High intensity joint directed impact increases risk (football, soccer)
Twisting forces (baseball) Moderate, regular running has no increased risk |
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Occupation and OA
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Jobs w/ repetitive use, highly physical labor increase risk of OA
15-30% of knee OA in men is work related |
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Genetics and OA
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50-70% of risk at some joints
Involved genes suspect to be polymorphism in ECM |
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OA of MCPs in young person?
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Check for hemochromatosis
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Major symptom of OA?
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Pain
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Treating OA
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Treating pain/joint failure
No disease modifying treatments Pain medication, steroids, glucosamine |
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What works to treat OA?
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Weight loss and strengthening
Joint replacement |
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Strengthening in OA
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OA leads to weak muscles
Pain is proportional to weakness Stronger muscles improve stability and reduce pain |
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Visosupplementation in OA
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Injection of hyaluronate into joints
Studies show modest improvement |
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DISH syndrome
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Diffuse Idiopathic Skeletal Hyperostosis
Ossification of anterior longitudinal ligament of spine Bony projections may cause esophageal dysphagia No SI, disc involvement |
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Polychondritis
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Inflammation of cartilaginous structures
Young patients Anti-collagen/elastin antibodies Saddle deformity Floppy ears/hearing loss/imbalance Respiratory stridor (trachea) Iritis, episcleritis Aortic insufficiency from dilated ring |
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Rheumatoid arthritis gender distribution
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Women>Men for articular symptoms
Men>>Women for extrarticular symptoms |
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Prevalence of RA
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0.1-1% of world's pop
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Rheumatoid arthritis presentaiton
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Morning stiffness
Many joints Both sides of midline Almost always involves hands Functionally limiting Feel systemically ill |
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RA histology
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Inflammation in synovium
Destroying all directions |
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RA arthroscopic appearance
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Frondy appearance of joint cartilage
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Physical exam in early RA
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Joint erythema, swelling, warmth, effusions
Loss of motion, flexion contractures Rheumatoid nodules Involving hands, wrists, shoulder, elbows |
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Physical exam in late RA
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Loss of joint motion--severe
Deformities Metacarpal subluxation and ulnar drift Swan necking of PIP Extraarticular disease |
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PE indicators of RA
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Wince with handshake
Inability to make claw hand inflammation of mcp, pip or flexor tendon |
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Diagnostic maneuver in RA
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Lateral MCP or MTP squeeze = pain
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RF
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Rheumatoid factor
IgM directed at Fc of IgG |
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anti-CCP
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Anti-citrinullated cylic peptide
Antibody against post-translational modification in proteins |
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Labs to get with suspected RA?
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CBC
RF Anti-ccp ANA LFTs Renal funciton |
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Lab findings in RA
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Slight low Hct
RF + 70-80% ANA + 10-20% |
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Best serologic marker for RA
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anti-CCP
70% sensitive, 95-99% specific As sensitive and more specific than RF Stable with disease (IgG) Predicts poor outcomes |
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Diseases that can have positive RF
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RA
Sjogren's Cryoglobinemia Juvenile Chronic Arthritis SLE Ankylosing spondylitis Infections Lung disease (interstital fibrosis, chronic bronchitis, silicosis) |
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RA joints on XRT
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Erosions and pock-marks in bones
Bare areas |
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Joint erosion progression in RA
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Rapid after onset of disease
90% in first two years Progression slows after that |
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Pathogenesis of RA
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Autoreactive CD4 T cells
These stimulate monocytes, synovial fibroblasts, macrophages which produced TNFalpha, Il-6, Il-1, MMPs FIbroblasts downregulate MMP inhibitors |
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Bone destruction in RA mediation
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Macrophages produce cytokines that activate osteoclasts
Synoviocyte makes MMPs, proliferate ---stimulate osteoclasts via RANKL |
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Genetics of RA
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HLA-DR beta chain
5 AAs : QKRAA or QRRAA Bind cittrulinated proteins Confers 5-20x risk Polygenetic disease |
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Major killer in RA
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Atheroscerotic heart disease
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Adaptive immune system and RA
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T-cell loss of tolerance seems to start things off, not important to progression of disease
Activated B cells may have role in immune complex mediated parts of disease |
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RA natural history
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Uncontrolled : 20-30% mortality at 10 years
20% on disability in 5 years 80% see earnings decline in 5 years |
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What is causing morbidity in RA
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Early -- inflammation
Late -- joint destruction |
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Treatment schema in RA
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Intervene ASAP with a disease modifying drug
Any disease modifying drug in first year makes a difference |
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DMARDs
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Prednisone
Hydroxychloroquine (avoid with eye troubles) Mtx Lefluonomide/SSZ TNF antagonist Rituximab CTLA-4 Ig - prevents the activation of T cells |
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Combination therapy in RA?
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Gets you a faster initial response
Seems to be similar to monotherapy in long run |
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Extra-articular RA manifestations
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RF-dependent, immune complex mediated
Rheumatoid nodules Vasculitis (nerve, digital infarct) Episcleritis/scleritis Lung fibrosis, effusions, nodules |
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Epi of extrarticular RA findings
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All declining except for nodules interstital lung disease
? 2/2 earlier RA treatment These processes usually show up after the joint disease has burned out |
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Rhematoid nodules
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Immune complex deposition leading to vascular compromise
Often at sites of trauma Macorophages / central necrosis Can evolve even with good control |
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RA effusions?
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low glucose
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Treatment of extraarticular symptoms of RA
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Cyclophosphamide
Steroids |
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CT consequences of RA
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Synovial compression of structures -- nerves -- Carpal Tunnel
Loss of ligamentous integrity -- Atlantoaxial subluxation |
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Felty syndrome
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RA
autoimmune neutropenia leg ulcers splenomegaly |
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Other extra-articular RA syndomes
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Sicca
Peripheral sensory neuropathy |
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Sequence of synovial inflammatory infilitrate
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Early on T, B, macros
Later PMNs |
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Poor prognostics in RA
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High RF, anti-CCP
Rheumatoid nodules Progressive disease of 1 year or greater Poor fnc HLA-DR shared epitope homozygosity |
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Lefluonomide
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Pyrimidine synthesis inhibitor
Used in RA |
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What can reverse course of RA
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Injectable gold
Cyclosporine Both shown healing of bone erosions Toxic |
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Caplan's syndrome
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RA-associated pneumocosis seen in patients with inhaled particle exposure (dust, silica, asbestos)
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Spondylarthritis
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Group of inflammatory conditions
Spinal and peripheral joint oligoarthritis Inflammation of attachments of ligaments and tendons to bones |
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Seronegative
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Producing a negative rxn on serum tests
Like RF and ANA |
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Ankylosis
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Stiffening of a joint
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Spondylos
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Vertebra
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Arthron
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Joint
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Seronegative spondylarthritis
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Inflammatory process involving spine
Axial arthritis |
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Ankylosing spondylitis
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Inflammatory process resulting in stiffening of spine
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Spondylarthritis =
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spondylitis
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Spondylarthropathies
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Ankylosing spondylitis
Psoriatic arthritis Reactive arthritis Enteropathic arthritis (Crohn's, UC) Juvenile AS Undifferentiated |
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Criteria for classifying spondyloarthritis
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Inflammatory spinal pain or synovitis (asymmetric or predominantly in lower limbs)
Plus one of these: Family history of SNSA Psoriasis IBD Urethritis, cervicitis, diarrhea recently Buttock pain alternating sides Enthesopathy Sacroilitis Sensitivity - 80%, Specificity 90% |
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Non-vertebral manifestations of spondyloarthritidies
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A -- aortic insufficiency, aortitis, heart block
N -- neurologic: AA subluxation, cauda equina K -- kidney - secondary amyloidosis S -- spine: cervical fracture, spinal stenosis, osteoporosis P -- pulmonary: upper lobe fibrosis, restrictive changes O - ocular : anteior uvetitis N -- nephropathy (IgA) D - discitis |
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Epidemiology of ankylosing spondylitis
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HLA-B27 strongly associated
0.6-1.3% of B27+ Ectomorph body type -- thin, medium height, long face, narrow chin Male 4: Female 1 |
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Diagnosing ankylosing spondylitis
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Sacroilitis on XRT
Reduced chest expansion or low back pain for >3 months improved by exercise and not rest or limited lumbar motin |
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What back pain makes you think of ankylosing spondylitis
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Onset of back pain before 40
Insidious onset Duration > 3 months Associated with morning stiffness Improvement with excercise |
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PE in ankylosing spondyiltis
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Occiput far from wall
Reduced chest expansion Schober test shows reduced excursion of lumbar spine on toe touch Pelvic compression yield pain at SI joint Patricks test Reduced flexion, abduction, external rotation of hip |
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Ankylosing spondylitis on XRT
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Bamboo spine
Thoracic and lumbar spine with vertebral squaring, osteopenia, ossification Preservation of disc spaces Sacroilitis |
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Aortitis increases risk of
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Aneurysm
Rupture |
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Treatment of ankylosing spondylisis
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NSAIDs
Sulfalazine TNFalpha antagonists Physical therapy |
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HLA-B27 association
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Common link of seronegative inflammatory conditions
95% of Europeans with AS 70% of reactive arthritis 60% of psoriatic arthritis 25% with peripheral psoriatic arthritis 70% with IBD and spondylitis 50% with acute anterior uveitis |
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HLA-B27 mechanism
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Unclear
Maybe B27 misfolds and provokes reaction B27 binds an artherogenic peptide B27 fails to clear an antigen quickly and results in a prolonged immune response |
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Psoriatic arthritis musculoskeletal features
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Asymmetric arthritis
Dactylitis/sausage digits Tenosynovitis Heel pain Sacroilitis Spondylitis |
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Psoriatic arthritis cutaneous featurs
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Psoriasis
Erythroderma Nail pitting Onycholysis Conjunctivitis, uveitis Valvular heart disease |
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Arthritis mutilans
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Loss of joint space
Seen in psoriatic arthritis |
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Psoriatic arthritis on xrt
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Erosive arthritis -- asymmetric
Pencil in cup deformity Arthritis mulitans Bony ankylosis Spurs/periosteal rxn Non-marginal asymmetric syndesmophytes Asymmetric sacroilitis |
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Treatment of psoriatic arthritis
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NSAIDs
Sulfasalazine Mtx/leflunamide TNFalpha antagonists ?prednisone PUVA - psoralen + UV |
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Reactive arthritis
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Reiter's - a subset
Seronegative mono/oligo asymmetric arthritis following: urethritis, cervicitis, infectious diarrhea |
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Reactive arthritis associated with
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Inflammatory eye disease
Sausage digit Enthesopathy Circinate balanitis - annular dermatitis of penis Oral ulceration Keratoderma blenorragica -- lesions resembling psorasis on palms/soles |
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Enteropathic arthritis
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Various IBDs
Asymmetric axial or peripheral Peripheral is large joints, late in disease, not B27 related Axial is more B27 related |
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Reactive arthritis course
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Most have relapsing course that declines in severity over a year/two
Resolution after single episode Least common is chronic course HIV + patients more likely to have fulminant course |
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What physical finding is predictive of joint involvement in psoriasis?
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Nail involvement
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Intestinal bypass associated with what CT disease?
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Mild arthritis in large joints
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Celiac disease and arthritis
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Variety of sites
Some evidence that gluten free helps |
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Normal synovial fluid
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Clear
Viscous <200 WBCs/mm3 |
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Inflammatory synovial fluid
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Cloudy
Thin Increased WBC |
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WBCs in synovial fluid
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per mm3
<200 normal 200-2000 -- non-inflammatory : OA 2000 - 99,999 -- inflammatory: RA, SLE, psoriatic, gout, pseudogout >100,000 - septic |
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Gout
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Monosodium urate monohydrate crystal deposition disease
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Monsodium urate monohydrate
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Final breakdown product of purine metabolism
Urate in body Uric acid in renal tubules |
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Normal serum urate levels
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<6.8mg/dL at 37 degrees
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Source of urate
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De novo synthesis of purines
Breakdown of nucleotides A bit from the diet |
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Uric acid excretion
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2/3 renal
via organic anion transporters in proximal tubule 1/3 gi |
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Hyperuricemia causes
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Overproduction (<10%)
HGPRT deficiency PRPP synthetase overactivity ETOH Lympho/myeloproliferative disorders Polycythemia Hemolytic anemia Psorasis Undersecretion (>90%) Chronic kidney disease Lead HTN Lactic acidosis Dehydration Diuretics Low dose salicylates Cylcosporine |
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Clinical features of gout
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Hyperuricemia
Acute monoarticular arthritis Tophi, chronic arthritis Nephroliathsis |
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Natural history of gout
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Stage I -- asymptomatic hyperuricemia
Stage II -- acute flares of arthritis, start as monoarticular and very intense, then more joints, less pain Stage III - flares on top of chronic arthritis |
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When do people transition from asymptomatic to symptomatic gout?
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1/7 do
men - 40 women - 65 do not start with stage I until menopause |
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Prevalence of hyperuricemia
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5% US adult males, more in France, more in hospitalized patients
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Gout risk and alcohol consumption
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Beer 4x, Alcohol 2x, Wine in moderation no increased risk
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Appearance of a gout inflamed joint
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Shiny
Erythematous Extremely sensitive |
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Gout crystals
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Monsodium urate monohydrate crystals are
Needle shapped, negatively birefrigent ( yellow ) |
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Birefringence
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Polarized light
If purple when parallel, postively birefringent If yellow when parallel, negatively birefringent |
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Acute gout pathogenesis
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Hyperuricemia and deposition of urate crystals in joint
Monocytes activated by TLR 2 and 4 NALP3 inflammasomes activated Il-1B released Results in TNFalpha, Il 6, 8, 10 production Upregulation of endothelial adhesion molecules Inflammation of joint |
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Acute gout cessation pathogenesis
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Activated monocytes mature into macrophages
Continue to ingest urate crystals Begin to elaborate TGFB anti-inflammatory Large proteins enter through permeable vasculature and coat crystals (ApoB) |
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Chronic arthritis of gout
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Degenerative arthritis
XRT - sclerotic erosion w/ overhanging lip |
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Goals of hyperuricemia therapy
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Get under 5
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Urate levels for tophi
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>8.2 = formation
<6.2 = reduction |
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Crystal of pseudogout
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Calcium pyrophosphate
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Crystal of Milwakee Shoulder Syndrome
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Basic calcium phosphate
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Provocative factors of gout attack
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Trauma
Alcohol Surgery Dietary excess Hemorrhage Drugs |
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Joints in an acute attack of gout
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MTP/podagra > instep > ankle > heel > knee > wrist > finger > elbow
15% are polyarticular |
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Chronic tophaceous gout
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10% of patients with gout
Total body uric acid pool is 20-30x normal 10 years after acute onset Most common sites: helix/antihelix of ear, olecrenon bursa, achilles |
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Most common extra joint complication of gout
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Renal disease
Nephrolithiasis in 10-25% 1000x RR Uric acid nephropathy -- ARF from deposition uric acid crystals in collecting system, seen in patients on chemo |
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Chondrocalcinosis
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Abnormal articular and periarticular cartilage
Assumed to calcium pyprophosphate crystal deposition |
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Pseudogout
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Acute attack of caclium pyrophosphate induced arthritis
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Who gets pseudogout?
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W>M
Older patients Twice as many people as get gout |
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Classification of pseudogout
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Sporadic/idiopathic - older women with osetoarthritis
Genetic Secondary to other disease hyperparathyroidism, hemochromatosis, hypophosphatemia |
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How does calcium pyrophosphate crystal disease present?
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Acute, sub-acute, or chronic arthralgias or arthritises
Mimics gout and OA |
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Favorite joint of pseudogout?
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Knee
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Calcium pyrophosphate crystals appearance
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Intracellular
Positive and weak birefringence Rhomboid crystals May be intermittent in chronic presentation Synovial fluid w/ <50K WBCs |
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Pathogenesis of calcium pyrophosphate crystal disease
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Pyrophosphate is made during many normal processes
Chondrocytes secrete into synovial fluid Crystals form Monocytes phagocytize and are activated by crystals |
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Which is more likely to have systemic symptoms, gout or pseudogout?
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Gout
Based on antigenicity of crystals |
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Treatment of pseudogout
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Symptomatic
Joint replacement |
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Osteoarthritic changes in wrong places?
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Consider pseudogout
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Most common of the crystal arthritises?
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Apatite arthropathy
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What are the basic calcium phosphates
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Hydroxyapatite
Octacalcium phosphate Tricaclium phosphate |
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What diseases are from basic calcium phosphate deposition
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Calcific periarthritis
Calcific tendonitis Arthropathies (pseudo-pseudogout) Milwaukee shoulder disease Erosive polyarticular disease |
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Milwaukee should disease
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Severe glenohumeral OA
"torn rotator cuff" Arthrogram shows subacromial dye |
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Basic calcium phosphate crystal appearance
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Not birefringent
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Effects of basic calcium phosphate crystals
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Increase MMPs
Decrease MMP inhibitors Stimulates PGE2 release Stimulates protease release Induce COX Increase TNFalpha, IL-1 Induce proto-oncogenes, mitosis |
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Mitosis induced in BCP disease results in?
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Osteophytes
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Basic calcium phosphate disease treatment
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Symptomatic
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Favorite joint of basic calcium phosphate disease?
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Shoulder
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Presentation of septic arthritis
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Acute
Monoarticular Pain at rest Structurally damaged joints are 10x more likely to get infected |
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Hepatitis and arthritis
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Prodrome of actue viral hepatitis
Prolonged with chronic |
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Why do you want arthrocentese a joint?
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Cell count/blood -- see how inflammatory, blood from trauma
Crystals Cultures |
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Synovial fluid in septic arthritis
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>75,000 cells
Mostly PMNs Appears opaque/purulent >3.0 protein <60 glucose |
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Predisposing factors for septic arthritis
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Joint disease
Trauma Systemic disease (DM, sickle cell, CVD, cancer) Immunosupression Prostetic jonts (espeically coag neg staph) IV drug use Endocarditis |
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Organism causing septic arthritis
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Kids -- staph, strep, salmonella
Adults -- neisseria, staph, strep |
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What joints affected by septic arthritis
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Kids -- knee, hip, ankle, elbow
Adults -- knee, hip, shoulder, elbow, sternoclavicular, ankle |
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Prognostic factors in septic arthritis
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Delay in diagnosis
Persistently positive joint cultures Prior arthritis (esp RA) Hips are worse than knees Pathogenesis of organism Host immune status IV drug users have better than expected outcomes with bad bugs |
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Therapeutic interventions effecting outcomes in septic arthritis
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Appropriate antibiotics
Effective drainage/debridement Physical therapy |
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What happens when septic arthritis diagnosis is missed?
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Joint destruction
Osteomyelitis |
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What organisms infect prosthetic joints?
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Coag neg staph -22%
Staph aureus -- 22% Gram neg bacilli - 25% Strep Enterococci Anaerobes |
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Early septic prosthetic arthritis
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Infection in first year
50% caused by Coag Neg Staph If sensitive organism, may try to save prothesisi If staph aureus/gram negative, remove prothesis |
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Treating an infected prosthetic joint
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Highest success rate with removal, antibiotics, delayed reimplantation (90%)
Next best: removal, antibiotics, immediate replacement (70%) Not good to just debride and give antibiotics |
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Risk of multiple unsuccessful debridements/revisions of infected prosthetic joint?
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Extensive bone loss
Requiring amputation |
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Appearance of infected bone
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Fuzzy on xrt
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When might it be okay to debride and retain joint?
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If removal is not possible
Early infection Avirulent organism Organism is sensitive to PO antibiotic that patient can tolerate Joint is not painful or loose |
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Do patients with prothesis need dental antibiotic prophylaxis
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No
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DDx of migratory arthritis
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Lyme
Rheumatic fever Gonorrhea |
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Fitz Hugh and Curtis syndrome
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Disseminated N. gonorrhea
Dermatitis Tenosynovitis Migratory polyarthritis |
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Chronic meningococcemia
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N meningitidis
Arthritis and dermatitis |
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Viruses associated with arthritis
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HepB,C,A
Rubella (vaccine) Parvovirus B19 Measles Mumps Enteroviruses Alphavirus HIV |
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Parvovirus B19
Four presentations |
Erythema infectiousum: Slapped cheek, macropapulary --->lacy reticular rash, fever, arthritis, anemia
-resolves over a few weeks Aplastic crisis in sickle cell Rheumatoid-like arthritis in adults persistant acute arthritis of small joints Hydrops fetalis |
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Rheumatoid like arthritis labs
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X-rays w/o erosions
RF - ANA - LFTs normal B19 IgM positive |
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Rubella symptoms
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Fever, malaise, lymphadenopathy, cold symptoms
Rash and arthritis follow Small joint, symmetric, looks like RA, can last for months |
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Post rubella vaccination arthritis
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Myalgias, arthralgias, arthritis, paresthesias
Begins 2 weeks post-vaccination Lasts one week (to one year) 15-20% of vaccine recipients |
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Blastomycosis
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Broad based budding fungi
Lung and bone trophism |
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Caseating granuloma on joint biopsy?
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TB
TB can infect anywhere |
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Dengue fever
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Flavivirus
Break bone fever Rapid diffuse spread of arthralgia/myalgia A. aegypti Increasing in prevalence because of changes to mosquite control |
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Chikungunya Fever
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Alphavirus
Arthritis with bent posture A. aegypti Widespread focal Currently limited to Africa |
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Where can you get dengue?
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Central/south america
India/SE Asia Parts of Africa Australia and NZ |
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Symptoms of prosthetic joint infection
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Joint pain - constant w/ increase when weight bearing
Fever - 40% Periarticular swelling -40% Wound -- 30% Can have normal ESR, WBC |
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Radiology in prosthetic joint infection
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Lucencies >2mm in bone
Migration of component Cement fractures Periosteal rxn Changes in components Takes 3-6 months of infection to develop xrt changes |
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Infectious causes of chronic monarticular or oligoarticular arthritis
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Bacterial -- lyme, whipple's, syph, nocardia
Fungi Mycobacteria Helminths |
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Catcher's Crouch Syndrome
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Post - rubella syndrome
Lumbar radiculopathy Pain in popliteal fossa, back of legs Pain is worse on arising in AM Patients stand in crouched position |
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Arm syndrome
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Post rubella
Brachial neuropathy Arm/hand pain Dyesthesias worse at night |
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Myopathy symptoms
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Fixed proximal muscle weakness
Premature fatigue Post-exertional ache, cramps, pains |
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Characterize myopathic vs neurpathic muscle weakness
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Myopathic is proximal and symmetric
--rest of neuroexam is normal Neuropathic proximal or distal, assymetric --other abnormalities on neuroexam |
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Muscle enzymes
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CPK
Aldolase SGOT (ALT) SGPT (AST) LDH May be positive in patients with myopathy |
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What causes a rise in CPK
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Asymptomatic (benign hyper CPK)
Myopathy Being an African American male Trauma (like an EMG) Exercise Carriers in glycogen storage, muscular dystrophy Drugs (EtOH, cocaine, statin, AZT) |
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Most common reason for having a high CPK?
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Asymptomatic hypercreatinephosphateemaia
AKA benign hyper CK |
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Inflammation of muscle on MRI
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White on T2, STIR images
This indicates edema, where you want to biopsy if you are looking to diagnose myopathy |
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Muscle biopsy histochemistry with O-red
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Increased red = increased lipid = abnormal
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Clinical features of idiopathic inflammatory myopathy
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Symmetric weakness of limb girdle/anterior neck
Muscle biopsy showing necrosis, fiber type grouping, inflammation Elevation in enzymes EMG - short polyphasic motor units, fibrillations, bizarre/repetitive discharges Dermatologic features |
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Are muscle enzymes helpful in idiopathic inflammatory myopathy?
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Usually elevated at some point during disease -- can help with diagnosis
LDH might be the best Do not correlate with disease activity |
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EMG in idiopathic inflammatory myopathy
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40% of patients have:
Fibrillation at rest Increased insertional activity Bizarre high-frequency repetitive discharges Polyphasic potentials of short duration and low amplitude |
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What are the idiopathic inflammatory myositises?
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Polymyositis
Dermatomyositis Myositis w/ associated collagen vascular disease Myositis w/ malignancy Inclusion body myositis |
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Polymyositis histology
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Cytotoxic CD8 cells invading throughout muscle fibers
Invading cells are memory cells (CD45RO) ICAM-1 expressed on muscle fibers |
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Dermatomyositis dermatologic features
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Heliotrope discoloration of eyelids
Periorbital edema Scaly, red rash over MCPs, PIP (gottron's) Rash over knees, elbows, medial ankle Rash on face, torso Capillary nail bed changes, periungal erythema |
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Histology of dermatomyositis
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Lymphocytic infiltrate concentrated around blood vessels
Perifascicular atrophy -- edges of fasicles are dying Capillary loss B lymphocytes, CD4s, plasma cells Complement ICAM-1 on vessels |
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Juvenile dermatomyositis
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Weakness
Rash plus Vasculitis Lipodystrophy Calcinosis |
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Amyopathic dermatomyositis
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Skin features w/o weakness
May develop into typical May be associated with malignancy |
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Inclusion body myositis
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Weakness can proximal or distal
Can be asymmetric Does not respond to steroids Lymphocyte infiltrate is transient Vacuoles Inclusions Mitochondrial abnormalities Amyloid depositis |
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Inclusion body myositis histology
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Triangular fibers
Fiber type grouping --looks a lot like neuropathic change |
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Self antibodies that might be positive in idiopathic inflammatory myositis
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Anti-synthestases (Anti-Jo-1)
Anti - Mi -2 Anti -SRP |
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Anti-synthetase syndrome
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Polymyositis > dermatomyositis
Interstitial lung disease Fever Arthritis Raynaud's Mechanic's hands Difficult to manage |
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Anti-SRP
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Polymyositis
Cardiomyopathy Also distal weakness Very poor prognosis |
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Anti-Mi2
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Dermatomyositis
Excellent prognosis |
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Prognostics in idiopathic inflammatory myopathy
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Autoantibodies change prognosis
Anti-SRP really bad (worse than cancer) Anti-Mi2 is good |
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Symptoms of mitochondrial myopathies in adults
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Exercise intolerance
Progressive proximal weakness Rhabdomyolysis Opthalmoplegia Lacticacidemia |
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Mitochondrial myopathy pathogenesis
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Genetic defect in mitochondrial protein
Gene can be either nuclear or mitochondrial |
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Prevalence of mitochondrial myopathies
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1 in 5000
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Histology of mitochondrial myopathies
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Ragged red fiber
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Hypokalemic myopathic syndrome
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Myalgias, cramps, fatigue
Flaccid, transient/persistent muscle weakness Increased CPK Vacuoles on biopsy Periodic paralysis w/ areflexia Rhabdomyolysis (myoglobinuria) |
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Electrolytes and muscles
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Anything that changes the concentration of
Na, Ca, Mg, Ph, K Can alter muslcle fnc Cause cramps, myalgias If severe: rhabdomyolysis and myoglobinuria |
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Steroid myopathy
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Normal enzymes
Proximal weakness Biopsy shows type 2 fiber atrophy Diagnosed by changing steroid dose |
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Cocaine myopathy
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Acute rhabdomyolysis w/ myoglobinuria
Elevated CPK in 30-50% even 3 months out |
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Illicit drugs that cause myopathy
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Cocaine
Amphetamines Pot Heroin PCP |
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Myopathy of heroin, ampetamines, pcp
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Rhabdomyolysis secondary to
ischemia agitation- convulsions coma w/ compression |
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Adult with dermatomyositis should be worked up for...
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Cancer
May be a paraneoplastic syndrome |
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Treating idiopathic inflammatory myopathy
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Steroids until symptoms resolve
Immunosuppresion (azathioprine) |
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Anti-Jo
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Antibody against
histydl-transfer RNA synthetase |
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Course of polymyositis
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70-80% 5 year survival
Less with pharyngeal involvement Usually recovery and relapse |
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Polymyositis Rheumatica
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Common disease in >50 yrs
Morning stiffness Muscle pain - shoulder, hip Fatigue Normocytic, normochromic anemia Elevated ESR No weakness Associated with temporal arteritis Dramatic response to steroids |
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Adult with dermatomyositis should be worked up for...
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Cancer
May be a paraneoplastic syndrome |
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Treating idiopathic inflammatory myopathy
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Steroids until symptoms resolve
Immunosuppresion (azathioprine) |
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Anti-Jo
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Antibody against
histydl-transfer RNA synthetase |
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Course of polymyositis
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70-80% 5 year survival
Less with pharyngeal involvement Usually recovery and relapse |
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Polymyositis Rheumatica
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Common disease in >50 yrs
Morning stiffness Muscle pain - shoulder, hip Fatigue Normocytic, normochromic anemia Elevated ESR No weakness Associated with temporal arteritis Dramatic response to steroids |
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Vasculitis
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Inflammation of blood vessel wall
Results in vessel wall destruction |
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Consequences of vasculitis
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Aneurysm formation
Occlusion of vessel Embolization from clotting on damaged vessel wall |
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Henoch-Schoenlein Purpura
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Small vessel vasculitis
Dependent skin rash Bloody diarrhea Glomerulonephritis Arthralgias IgA, PMNs in vessel wall |
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Henoch-Schoenlein purpura aka
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Hypersensitivity vasculitis
Skin picture is similar to drug reaction vasculitis |
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Skin signs of vasculitis
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Palpable purpura
Ulcers -- esp w/ pointed edges Nodules Ischemic digits Livido reticularis (dilation of dermal vessels) |
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Small vessel vasculitis effects?
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Arterioles
Capillaries Post-capillary venules |
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Polyarteritis nodosa is associated with?
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Hepatitis B infection -- almost always
Decreasing in incidence because of better HepB treatment Similar process with RA, SLE, Kawasaki's |
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Polyarteritis nodosa
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Medium size vessel vasculitis
Neutrophilic, fibrinoid necrosis Gut ischemia, coronary aneurysms, renal artery HTN, large ulcers, mononeuritis |
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When to think vasculitis should be on DDx?
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Palpable purpura
Diseases of the old in the young Glomerulonephritis Mononeuritis multiplex Systemic disease w/o cancer, infection Multisystem illness |
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Causes of secondary vasculitis
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Connective tissues diseases
Infection (HepB,C, parvovirus, zoster, subacute bacterial endocarditis) Drug induced Cancer Cardiac mxyoma Cryoglobulins |
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Etiology of idiopathic/primary vasculitis
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Immune complex deposition or formation on vessel walls
Inflammatory rxn Location likely determined by adhesion molecules, turbulence, hydrostatic pressure Probably a stimulus brings on, but we don't know what yet |
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Wegner's granulomatosis
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Small vessel vasculitis
Vasculitis and extravascular granuloma formation in: Upper airway -- sinus, nose, ears Lower airway -- lungs, trachea Kidney --focal/segmental glomerulonephritis c-ANCA positive |
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Wegner's granulomatosis presentation
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Epistaxis
SOB Glomerulonephritis Systemic symptoms |
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ANCAs
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Anti-neutrophil antibodies
cANCA - cytoplasmic staining - usually against serine protease 3 pANCA - perinuclear staining - myeloperoxidase is antigen |
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Microscopic polyarteritis/polyangitis
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Small vessel vasculitis
pANCA positive Skin, kidney, lungs Diffuse alveolar hemorrhage syndrome |
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Churg-Strauss syndrome
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Small vessel vasculitis
Patients with asthma, atopy Pulmonary infilitrates, cardiac, neurologic Blood eosinophilia |
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Takayasu's vasculitis
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Inflammation of aorta and branches
Fever, malaise, weight loss, ischemia symptoms initially Progressive vascular occlusion Pulse-less disease Asian predominance |
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Giant cell arteritis
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Large vessel vasculitis
Usually extracranial arteries Mononuclear cell infiltrate destroys elastic lamina of musclar arteries Headache, facial pain, ischemic vision loss Most common vasculitis in >55 Associated with PMR Very sensitive to prednisone |
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Gold standard for diagnosing vasculitis?
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Biopsy
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Bechet's
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Vasculitis of arteries and veins
Painful oral/genital ulcers, thrombophlebitis, retinal vasculitis, anterior eye disease, encephalitis, rash |
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Cryoglobinemic vasculitis
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Cold precipitating antibodies
Small vessel vasculitis Glomerulonephritis Strongly associated with HepC |
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Buerger's disease
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Thromboangitiis obliterans
Obliterative arterial and venous disease of hand and feet Can cause digit loss Related to smoking May be a vasculopathy |
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Treating vascultis
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Remove precipitant
Use anti-inflammatories for quick control of inflammation Consider cyclophosphamide, azothioprine, Mtx Treat sequelae of inflammation/treatment |
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Most common vector born disease in US?
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Lyme
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What transmits lyme to people?
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Ticks -- deer tick
Tick's with black legs |
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Areas with highest risk of Lyme disease
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Southern NE/Midatlantic, esp seacost
Wisconsin Really -- near deer herds |
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Bannworth's Syndrome
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Erythema chronicum migrans
Ixodes ricinus tick bite Meningioradicularneuritis Chronic lymphocytic meningitis Radicular pain in dermatome of tick bite |
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Borrelia garinii
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European strain
Neuroborreloisis |
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Borrelia afzelli
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Acrodermatitis chronica atrophicans
Borrelial lymphocytoma |
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Spirochetes cause what type of illnesses?
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Chronic relapsing ones
Lyme Relapsing fever Syphilis |
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Borrelia bergdorferi
Symptoms and virulence |
Symptoms: arthritis, neurologic, cardiac
Virulence comes from its lasting ability -- lots of plasmids for different out structures Migration -- when blood hits midgut, bacteria changes it outter surface to be trophic to salivary glands--move from tick to human |
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Lyme disease Stage I
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Erythema chronicum migrans
Flu-like: fever, malaise, headache, conjunctivitis, fatigue, arthralgias, lymphadenopathy |
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Lyme disease Stage II
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Arthritis -- 60%
Neurologic - 10% menigoencephalitis cranial neuropathies (Bell's) radiculopathy Cardiac - 5% heart block -resolves usually myocarditis |
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Lyme meningitis vs viral meningitis
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Lyme is -- longer course, afebrile, few PMNs in CSF
Accompanied by CN palsy, papilledema, rash |
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Lyme disease Stage III
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10% of patients
Chronic arthritis (mono or pauciarticular) Tertiary neuroborrelosis chronic encephalomyelitis persistant polyneuritis Acrodermatitis chronica atrophicans localized sclerotic/atrophic lesions |
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What do people treated for Chronic Lyme disease have?
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No clinical evidence of Lyme - 70%
Untreated stage III infection - 20% Post-lyme disease syndrome - 10% fatigue and arthralgias -- self-limited |
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Serologic positivity in Lyme disease
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Delayed, not specific
Rarely positive in stage I Unlikely negative in stage III But watch out for false positives |
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False positives in Lyme serologies
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Cross rxn with other spirochetes
Past exposures Sera with high titer of autoantibodies |
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False negatives in Lyme serologies
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Early antibiotic treatment may be serongative even in symptomatic relapse
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How is lyme diseae serology done
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A number of positives on western blot
More IgGs needed for positive test |
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What is the lyme disease reservoir
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Mice
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Treating lyme
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Usually doxycycline
In meningitis, heart block, severe arthritis -- use ceftriaxone Can also use amox, cefuroxime, pen G |
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Preventing Lyme disease
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Prompt removal of tick - with tweezers on mouthparts
Skin repellents (DEET, N,N-dietyl-m-tolulide) Clothing repellants - permanone, permethrin |
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Babesiosis
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Hemolytic anemia
Fever Splenomegaly Intracellular parasite transmitted by same tick as Lyme disease |
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Erlichiosis
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Tick born bacterial infection
Bacteria: erlichia -obligate intracellular: moncytes Vector: amblyoma americam Reservoir: deer Headache, fatigue, muscle aches Monocytic reaction |
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Anaplasmosis
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Tick born bacterial infection
Bacteria: Anaplasma phagocytophilium obligate intracellular: PMNs Tick: Ixodes scapularis Reservoir: mouse Fever, HA, chills, myalgia |
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Southern Tick Associated Rash Illness
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Amyblomma americum
Borrelia lonestarii Bulls eye rash Flu-like illness |
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Most commonly injured joint?
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Knee
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First degree ligament injury
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Local tenderness
No instability on stress |
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Second degree ligament injury
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Joint opens on stress, but there is an endpoint
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Third degree ligament injury
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Joint opens on stress without endpoint
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Mechanism of MCL injury
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Direct blow to lateral knee
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Physical findings of MCL injury
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Pain with valgus stress
Laxity of medial structures when lateral (valgus) stress applied (in 30 flexion) Associated with ACL, meniscal injury |
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Treating MCL injury
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Grd1 - symptomatic
Grd2- knee brace Grd3-- knee brace vs surgical repair |
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LCL injury
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Uncommon
Vargus stress (hit from medial side) Symptoms opposite of MCL, treatment same Check for common fibular nerve injury |
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ACL anatomy
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Two bundles (anterolateral, posteriomedial)
Tense throughout ROM of knee Poor healing capacities |
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Mechanism of ACL injury
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Rapid acceleration, decell, and/or rotation on a planted foot
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History in ACL injury
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Audible pop
Immediate pain Rapid swelling |
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PE in acute ACL injury
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Hemarthrosis
Limited ROM Lachman, anterior draw, pivot shift tests all positive |
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Radiography in ACL injury
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X-ray -- lateral capsular damage = ACL tear
Confirm tear by MRI |
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ACL tear treatment
|
Non consensus on reconstruction vs nonoperative management
Reconstruction is arthroscopic guided graft form patellar or hamstring Includes: age, athleticism, rehab motivation, associated injuries |
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ACL rehab
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Up to a year of intensive muscle retraining
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PCL injury
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Much less common than ACL
Often a dashboard injury Rapid posterior acceleration of leg |
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Diagnosing PCL injury
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Often missed
Hyperextensible, positive posterior drawer sign MRI confirms |
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PCL treatment
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Nonoperative for isolated PCL injury
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Knee dislocation definition
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Grossly unstable knee
Disruption of both cruciate and at least one collateral |
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Treatment of knee dislocation
|
Often reduce spontaneously
Arteriogram in all cases 2/2 High risk of neurovascular damage (popliteal, peroneal) Surgery if: open, artery damage, unreduceable --either immobilize or repair each ligament |
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Meniscus anatomy and fnc
|
c shaped, biconcave fibrocartilagenous discs
outer 1/3 is vascular Reduce condral stress by 4x |
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Mechanism for meniscal injury
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Flexion, rotation
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Symptoms of meniscal injury
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catching, swelling, giving way
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Meniscal injury PE
|
Joint line tenderness
Effusion Incomplete extension + McMurray's |
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McMurray's sign
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Passive external rotation of knee with valgus pressure
Click/pain -- mensical injury |
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Imaging in meniscal injury
|
MRI usually diagnostic
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Treatment of meniscal injury
|
Observation
Arthroscopic meniscal repair or removal |
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Patella function
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Increase force of extension
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Patellofemoral joint vasculature, stress
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Blood supply from geniculate arteries
Contact stress increases with flexion |
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Symptoms of patellofemoral joint dysfunction
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Giving way
Pain on inclines Pain with prolonged flexion Creptiation Swelling |
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Patellofemoral joint dysfunction
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peripatellar tenderness
pain with patellar compression |
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Treatment of meniscal injury
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Observation
Arthroscopic meniscal repair or removal |
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Patella function
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Increase force of extension
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Patellofemoral joint vasculature, stress
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Blood supply from geniculate arteries
Contact stress increases with flexion |
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Symptoms of patellofemoral joint dysfunction
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Giving way
Pain on inclines Pain with prolonged flexion Creptiation Swelling |
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Patellofemoral joint dysfunction
|
peripatellar tenderness
pain with patellar compression quad atrophy apprehension of instability |
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Patellofemoral syndrome
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AKA chondromalacia patella
Pain or crepitation in knee at PF joint Effusion Positive patellar compression test Commonly see in adolescent females Treated non-operatively with quad strengthening |
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Patellar disolcation
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Occurs with trauma, direct or indirect
Patella displaces laterally Treat by reduction, immobilization or operative repair/stabilization for recurrent dislocations |
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Patellar tendinitis
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Pain at inferior pole of patella
From repeated stress on insertion point "Jumper's knee" Conservative treatment |
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Osteochondritis dissecans
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Separation of avascular fragment of bone/cartilage from underlying normal bone
Medial femoral condyle is most common site Seen in adolescents Treatment is conservative is fragement is stable, reattach if unstable Isolated cartilage disease can be treated with chondrocyte transplant |
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Osgood-Schlatter's disease
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Pain at tibial tuberosity from patellar tendon traction
Adolescents XRT shows fragmentation at tuberosity Treated with activity modification, ossicle may need revision later in life |
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Sindig-Larsen-Johannson disease
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Pain and fragmentation at inferior pole of the patella
Treated symptomatically |
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Bipartate patella
|
Incidental finding
Not a fracture 50% bilateral No treatment needed |
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Shoulder joint movement
|
As glenohumeral joint abducts, scapulothoracic abducts
2:1 motion of glenohumeral to scapulothoacic |
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Shoulder stability determinants
|
Bony congruity
Ligamentous stability Dynamic muscular stability |
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Most common fracture site in childhood
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Clavicle
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Acromioclavicular joint injuries
|
Graded based on degree of joint separation
Operative only in higher degrees |
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Diagnosing acromioclavicular joint injuries
|
XRT on cephalic tilt view, comparison view, stress views
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Acromioclavicular joint injuries
|
If they cause chronic pain
--excise the distal clavicle and possible repair the coracoclavicular ligaments In kids w/ injury to medical clavicle -- worry about physis injury |
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Acute glenohumeral instability -- anterio
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Anterior >>> Posterior
Anterior -- abduction and external rotation recurrence rate inversely proportional to age axillary nerve damage rotator cuff injures in elderly Treat with immobilization |
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Posterior glenohumeral disslocation
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Often missed diagnosis
Need axillary xrt to diagnose Can be seen after ECT, seizures Treat with closed reduction and immobilization in external mobilization |
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Chronic recurrent anterior glenohumeral instability
|
Acute dislocation easily diagnosed
Recurrent subluxations more difficult Bankhart lesion- labrum tear Hill-sachs lesion -- capsular laxity -- multidirectional instability |
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PE findings with chronic anterior glenohumeral instablity
|
Apprehension sign -- to abduction and external rotation
Sulcus sign -- anterior and inferior instablity --should drop |
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Treatment of chronic anterior glenohumeral instability
|
physical therapy
operative -- arthroscopic or open |
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Recurrent posterior glenohumeral instability
|
Check for habitual or voluntary dislocators
--psych issues Aggressively PT, surgery does not work well |
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Rotator cuff injury symptoms
|
Night pain
Painful arc of motion Restriction of motion |
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Rotator cuff related injuries
|
Rotator cuff tendinitis
Subacromial bursitis Impingement syndrome |
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Impingement syndrome
|
Coracoacromial arch encroaches on rotator cuff mechanism
Acromial shape/morphology predictive Diagnosis by history, physical Treatment based on degree of rotator cuff injury (tendinitis vs tear) Acromioplasty and rotator cuff surgery can be done arthroscopy |
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What shape of acromion is most at risk for impingement syndrome?
|
Hooked
20% of the population |
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Calcific tendinitis
|
Usually self-limiting
Acute flares of tendinitis in patients with calcific tendinitis may be 2/2 reabsorption of deposits Consider needle aspiration if acutely painful |
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Adhesive capsulitis
|
Contracted, thickened capsule drawn tightly around the humeral head
Limited in passive and active ROM Etiology unknown association with diabetes and liver disease Aggressive physical therapy, stretching |
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|
Acute rotator cuff tear
|
Usually supraspinatous
Acute tears rare Elderly with anterior dislocation Normal passive ROM, limited in active ROM Consider surgery |
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Chronic rotator cuff injury
|
End stage impingement syndrome
Surgical treatment usually relieves pain but may not restore function atrophy of muscles |
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End stage rotator cuff injury on xrt
|
Retraction of the humeral head up through hole in rotator cuff
No longer articulating with glenoid |
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Long head of the biceps rupture
|
Retraction of muscle -- popeye muscle
Lose a little supination Otherwise functionally intact |
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Biceps tendon instability/tendinitis
|
Instability associated with large rotator cuff tears
Tendinitis associated with impingement syndrome |
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Wolff's Law
|
Bone develops a structure most suited to resist the forces acting upon it
Application of force -- bone deposition Removal of force -- bone resorption Together combine to remodel bone |
|
|
Growth plate
|
Longitudinal growth follows a cartilaginous template
Reserve zone Zone of proliferation Zone of hypertrophy Primary spongiosum |
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Lamellar bone
|
Hypocellular
Collagen fibers in parallel Slow growing but strong |
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How does compact cortical bone develop?
|
The forces of standing, walking, weight bearing result in osteon formation
|
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Where is cancellous bone found?
|
Marrow areas
|
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|
Metabolic bone unit
|
Osteoclast
Osteoblast |
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Woven bone
|
Cellular
Large osteophytes Non-parallel collagen fibers Not as strong as lamellar bone Can be layed down quickly without cartilage precursor Pathologic in adults |
|
|
Problems observed in children with musculoskeletal disease
|
Retrogression of developmental milestones
Weight/height retardation Localized growth abnormalities Delayed secondary sexual characteristics |
|
|
Juvenile Idiopathic Arthritis
|
Chronic persistent arthritis in 1 or more joints >6 weeks in child <16 year with other causes excluded
Most common connective tissues disease of childhood |
|
|
Systemic JIA
|
Fever, rash, variable arthritis, hematologic symptoms, ascites
Elevated ESR/CRP Autoantibody negative M=F |
|
|
Pauciarticular JIA
|
Onset<3:
Female > Males ANA + Knee, ankle, wrist Chronic Uveitis 6-10 : Male = Female 11-16: Male > Female Lower Limb Enthesitis Acute uveitis |
|
|
Polyarticular JIA
|
0-3
F>M Small/Lg joints Tenosynovitis 6-10: F>M 11-16: F>>M RF+ Symmetric Erosive |
|
|
Juvenile idiopathic arthritis histology
|
Villous hyperplasia of synovium
Hypervascularity Infiltration of lymphocytes and macrophages Pannus |
|
|
Potential consequences of synovitis in JIA
|
Joint contracture
Erosions Deformity and functional limitations |
|
|
JIA and growth
|
Epiphysial overgrowth
Stimulated by growth factors of inflammation Initial effected joint limb is longer Premature closure leads to shorter limb overall |
|
|
Microganthia
|
Undersized jaw
One possible feature of JIA |
|
|
JIA and spine
|
Mid-apophyseal joint involvement common
|
|
|
Uvetitis risk
|
Blindness if untreated
band keratopathy, cataracts Treatable with ocular steroids |
|
|
Which kids with JIA really need to be screened for uveitis?
|
Young kids (<7) with ANA positive mono or oligoarticular disease
System cases have low risk Everyone else is between |
|
|
What do you look for on a joint exam?
|
Swelling
Tenderness Limitation of motion |
|
|
Benign juvenile hyper mobility syndrome
|
Cause of arthralgias
|
|
|
Managing JIA
|
Diet w/ emphasis on protein, calories
Splinting to prevent deformity esp at night Physiotherapy for joint mobility and muscle function NSIADs, DMARDs |
|
|
Mortality in systemic JIA
|
15% after 15 years
|
|
|
How many patients with polyarticular JIA will continue you have symptoms into adulthood?
|
50%
Likely to decline with biologics |
|
|
Chronic pain prevalence
|
10-15% of pop report widespread chronic pain
20-25% chronic regional pain Clusters w/ fatigue, unrefreshed sleep, cognitive difficulties, mood disturbance |
|
|
Peripheral pain
|
Nociceptive
Damage/inflammation in tissues Responds to NSAIDs, procedures |
|
|
Neuropathic pain
|
Damage/dysfunction of peripheral nerves
Response to peripheral/central agents Ex. Diabetic neuropathy |
|
|
Central pain
|
Non-nociceptive
Disturbance in pain processing Diffuse hyperaglesia, allodynia Responsive to neuroactive agents, changes in NTs in brain Ex. Fibromyalgia, IBS, tension HA |
|
|
Workup of nonarticular pain
|
Mechanism: peripheral, neuropathic, central
Etiology: traumatic, infectious, systemic, somatoform |
|
|
Fibromyalgia
|
Chronic, widespread pain syndrome occuring in the absence of demonstrable inflammatory or systemic disease
My co-exist with autoimmune, inflammatory or thyroid disease 11 of 18 tender points, >3 months |
|
|
Myofacial pain syndrome
|
Localized non-articular chronic pain chronic pain with trigger points
|
|
|
Trigger points
|
Local areas of muscle tenderness
On palpation exhibit taut band consistency w/ twitch response and referred pain Common: suboccipital, trapezius, levator, piriformis Tender points do not produce referred pain |
|
|
Pathogenesis of fibromyalgia
|
Deficiency of NE and 5HT, excess of excitatory NTs
Lack of sleep, exercise increase pain Cognition about pain effects its perception Infectious, physical, emotional trigger produces central and periperal changes in muscle conditioning, sleep, pain |
|
|
Pain sensitivity
|
Threshold for producing central sensation of pain from peripheral inputs
Set by genetics, modified by neurohumeral factors, neural plasticity, emotion, stress |
|
|
Prevalence of fibromyalgia
|
3.4% of females
0.5% males Prevalence increasing with age |
|
|
Sleep disturbance in fibromyalgia
|
EEG changes
Stage IV sleep waves intruding on Stage III |
|
|
Mood disorders in fibromyalgia
|
25-35% incidence of DSM diagnoses
Depression is most common Fibromyalgia and chronic illness increase lifetime risk of depression |
|
|
What is increased in CSF of patients with fibromyalgia?
|
Substance P
|
|
|
Treating fibromyalgia
|
SNRIs, NSRIs, TCAs, anticonvulsants
Tramadol, SSRIs, gamma hydroxybuterate, Dopamine agonists Not: opiods, NSAIDs, corticosteroids |
|
|
FDA approved medications for fibromyalgia
|
Duloxetine
Milnacipran Pregabalin (voltage gated calcium channel binder in CNS) |
|
|
Nonpharmacologic therapy in fibromyalgia
|
All show effective
Exercise Biofeedback Hypnotherapy Acupuncture |
|
|
SLE Epi
|
1 in 1000
Usually presents in 20-30s F>M 8:1 More common in blacks, hispanics, polynesians than whites |
|
|
Genetics of SLE
|
Inherited complement disorders (C2, C3) often seen in certain types
Polygenetic 50% twin concordance Higher rates in first degree relatives, also antibodies without disease in relatives Most people show no genetic predisposition |
|
|
Pathogenesis of SLE
|
Autoreactive T and B cells
Exposure to autoantigen Production of autoantibodies Failure to clear immune complexes Increased delivery of immune complexes to spleen Overwhelming RES system in spleen Deposition of autoantibodies in tissues Direct damage of tissue by autoantibodies (cytopenias) |
|
|
What reduces ability to clear immune complexes (enhances progression of SLE)?
|
Complement deficiencies
Polymorphisms in membrane complement receptors Different Fc gamma receptors |
|
|
How does immune complex cause damage?
|
Intense local inflammatory response to fixed immune complex
Complement mediated |
|
|
What do immune complexes damage in SLE?
|
Kidney
Skin Blood vessels Pleura ?choroid plexus |
|
|
What do autoantibodies directly damage in SLE?
|
Blood cells (cytopenias)
? brain --anti-neuroal ? kidney -- membraneous |
|
|
Lupus band test
|
Immunoflorescence of skin showing Ig and complement deposition at DE jct
Not specific |
|
|
Non-specific symptoms of lupus
|
Fatigue -- 80-100%
Fever -- 80%, w/ flare Arthritis that looks like RA but is non-inflammatory and non-erosive Morning stiffness and myalgias |
|
|
Mucocutaneous symptoms of lupus
|
85% of patients have some, present symptom in >half
Butterfly/malar rash -- erythematous, edematous eruption on both cheeks and bridge of nose. usually transient Discoid lesion - Hyperkeratotic with central atrophy, central loss of pigmentation 20-30% patients Alopecia - 70% Mucosal ulcers -40% |
|
|
SLE and renal disease
|
Almost all have some
<half clinically evident Mesangial disease -- proliferation of mesangial cells, IgG and complement deposition, usually asymptomatic, most common Focal proliferative lupus nephritis-- focal glomerular inflammation, treatable Diffuse proliferative nephritis- Most severe, can lead to renal failure Membraneous nephritis-- Pathologically thickened basement membrane, no proliferation, may have lots of proteinuria, but not bad prognosis |
|
|
Diffuse proliferative nephritis in lupus
|
Most severe form of lupus kidney disease
Life shortening Associated with anti-dsDNA HTN, renal insufficiency, nephrotic syndrome Hematuria, casts Epithelial crescents, wire loop lesions, hyalin thrombi, fibrionoid necrosis, endothelial proliferation |
|
|
SLE and heart
|
at least 20% of patients
Pericarditis common Myocarditis with focal T infilitrates and conduction abnormalities Verrucous endocarditis (Libman-Sacks) uncommon CAD - disease and treatment play roles |
|
|
Neuropsychiatric disease and SLE
|
25-75% of patients have disease
Functional: Depression, mania, mood swings, adjustment disorder, anxiety (about dependency, disfigurement) Organic: Seizures (15-20%) CVA - related to thrombosis or vasculitis Transverse myelitis 24% pyschosis |
|
|
Most common anemia in SLE
|
Anemia of chronic disease
Not autoimmune |
|
|
SLE and hematologic
|
Cytopenia -- anemia, leukopenia, thrombocytopenia
Lympadenopathy -- 50% Splenomegaly - 20% |
|
|
Lupus anticoagulant
|
Prothrombotic autoantibody to prothrombin activator complex
Prolongs PTT, not restored by mixing Associated with thrombotic disease, thrombocytopenia, recurrent fetal loss |
|
|
Lupus and clotting
|
Autoantibodies to 8, 9, 11, 12, 13 sometimes seen
Lupus anticoagulant is also sometimes present -- this is prothrombotic |
|
|
Diagnosing lupus
|
4 or more of these
Malar rash Discoid Rash Photosensitivity Oral ulcers Arthritis Serositis Hematologic disorder Renal disorder Antinuclear antibodies Immunologic (anti-dsDNA, antiSM or BFP) Neurologic |
|
|
ANA and lupus
|
Antibodies to nuclear material
Sensitive, but not specific test Used to confirm, not screen for lupus |
|
|
SLE and anti-dsDNA
|
Positive in 20-70% of SLE
Specific Can fluctuate with disease intensity |
|
|
SLE and anti-SM
|
Antibody against a small nuclear ribosomal protein
Positive in 20-30% of SLE Specific |
|
|
Disorders with positive ANA
|
Lupus
Scleroderma Paciarticular JIA Sjogren's Mono Subactue bacterial endcarditis Malaria Leprosy Neoplasms Chronic hepatitis Hydralazine, procanamid Age |
|
|
Anti-SS-A
Anti-Ro |
SLE, Sjogren's, subacute cutaneous lupus, neonatal lupus
|
|
|
Histone antibodies and lupus
|
Positive in most drug induced lupus
Positive in half of idiopathic lupus |
|
|
What autoantibodies are elevated in Sjogren's syndrome
|
60%
anti-SS-A anti - SS - B (Ro and La) |
|
|
Treating SLE
|
Done by symptom
Steroids for serositis, heme, renal, CNS Seizures with anti-seizure meds Skin with antimalarias Rituximab? |
|
|
Discoid lupus
|
Skin manifestation only
50% are ANA positive Treat with hydroxychloroquine and topical steroids |
|
|
Drug induced lupus
|
Hydralazine, isoniazid, aldomet, dilantin, procanamide
Others ANA, histone positive Arthritis, serositis, hepatomegaly Other onset than idiopathic Rarely hematologic, CNS, renal No immune complexes Withdraw drug |
|
|
Lupus in pregnancy
|
Normal fertility
Increased fetal loss (25-30%) -worse with renal disease Clotting is probably mechanism 50% of patients have lupus flares during pregnancy |
|
|
Antiphospholipid antibody syndrome
|
Thrombotic disease -- arterial and venous
Recurrent fetal loss Thrombocytopenia Lupus anticoagulant or anti-cardiolipin or false positive syphilis test Anticoagulation for life 25% of SLE patients, others without SLE also Lividio reticularis |
|
|
Cryptic arterial thrombosis
|
Antiphospholipid syndrome
Cancer Hyperhomocystinemia |
|
|
Catastrophic antiphospholipid syndrome
|
Multiple simultaneous clotting events leading to multi system organ damage
|
|
|
Jaccoud's Arthropathy
|
SLE
Tendon laxity causes hand to look like RA, but can be passively restored (not erosive) |
|
|
What does lupus renal disease look like on anti-Ig immunofluroescence?
|
Spotty, granular
|
|
|
Major determinants of m and m in SLE?
|
Renal disease
CNS disease |
|
|
New ideas in lupus etiology
|
fragile keratinocytes -- increase apoptosis leads to available autoantigens
Endogenous TLR activation IFN overexpression EBV antigen for autoantibodies |
|
|
Normal healthy young women with ANA +
|
5%
|
|
|
Treating SLE renal disease
|
Cyclophosphamide
and Corticosteroids |
|
|
New drug for SLE
|
Belimimab
|
|
|
Late complications with SLE
|
Osteonecrosis - 14% of patients -avascular necrosis of hip
Osteoporosis - 65% -- prednisone use Premature atherosclerosis - 6-10%, associated with duration of disease - corticosteroids increase risk factors |
|
|
Scleroderma
|
Disease of too much collagen deposition
Pathogenesis unknown involves immune system, vasculopathy |
|
|
Systemic sclerosis epi
|
15/million annual incidence
250/million prevalence Female 4: 1 Male Peak prevalence 30-50 |
|
|
Connective tissue changes in systemic sclerosis
|
Increased fibroblast deposition of Types I and III collagen in interstitum
Collagen is normal Likely an over-response to cytokine stimulation (TGFbeta, IL-4) Leads to tightened, bound skin, limiting fnc |
|
|
Vascular changes in systemic sclerosis
|
Raynauds
Swelling, hypertrophy, endothelial disruption Thombi |
|
|
Immunologic changes in systemic sclerosis
|
Early in disease infilitrate of CD8 and 4 T cells into interstitum of skin
Increase of many cytokines (Il 2, 4, 6, 8, TgfB, TNFalpha) |
|
|
Histology of skin in systemic sclerosis
|
Increased compact collagen in reticular dermis
Hyalinizaiton and obliteration of small vessels Loss of normal dermal appendages Thinning of epidermis Increase in melanin |
|
|
Histology of blood vessels in systemic sclerosis
|
Concentric proliferation and mucoid thickening of intima
Little involvement of media Fibrosis of adventitia Medium sized |
|
|
Systemic sclerosis cutaneous features
|
Sclerodactyly
Telangectasias Calcinosis Raynauds |
|
|
Progression of skin disease in systemic scleroderma
|
Edema (bilateral, pitting)
Thickening and tightening of skin Taut, shiny, indurated skin Contraction of fingers Loss of normal wrinkles and folds |
|
|
Telangectasias in scleroderma
|
Fingers
Nail folds Palms Face Lips |
|
|
CREST
|
aka limited cutaneous scleroderma
Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangectasias Skin disease does not get to axial regions |
|
|
GI tract and systemic sclerosis
|
80% have esophageal dysmotility in distal 1/3. Solid food dysphasia
Small bowel pseudo-obstruction and dilation Colon - wide mouth diverticuli |
|
|
Pulmonary and systemic sclerosis
|
100% of patients have pulmonary fibrosis
T cell and Il-4 mediated Bibasilar interstitial fibrosis - where pulmonary arterial vasculature as lost a muscularis Systemic disease Pulmonary HTN - limited disease Pleural disease Increased rates of alveolar cell carcinoma |
|
|
Sytemic sclerosis and kidney
|
Mild diastolic HTN
Malignant HTN (scleroderma renal crisis) concentric, intimal proliferation of interlobular arteries Unpredictable but usually in first 3 years |
|
|
Systemic sclerosis and muscles
|
Looks a lot like polymyositis/dermatomyositis
|
|
|
Diagnosis systemic sclerosis
|
Thickening, tightening of skin on fingers plus 2 of
Sclerodactyly Digital pitting scars, loss of substance of finger pad Bibasilar pulmonary fibrosis |
|
|
Auto-antibodies in systemic sclerosis
|
anti-centromere
anti - Scl-70 30% sensitive, highly specific in systemic sclerosis 70% of CREST patients have anti-centromere |
|
|
Diffuse systemic sclerosis
|
Generalized skin changes not limited to hands
More likely to have extensive kidney/lung involvement anti-Scl-70 if anything Rapidly progressive |
|
|
Treating scleroderma
|
Not much works
ACE-inhibitors control renal disease Calcium channel blockers help with Raynauds Cyclophosphamide may slow progression down |
|
|
Pulmonary artery HTN and scleroderma
|
Seen in limited form
Can be fatal Treated like idiopathy pulmary artery HTN calcium channel blockers prostaglandins sildenafil |
|
|
Survival in systemic sclerosis
|
Limited and diffuse combined
60-70% at 5 years 40-50% at 10 years |
|
|
Most common causes of death in systemic sclerosis
|
Pulmonary
Fibrosis in diffiuse HTN in limited This is because of ACE inhibitors, before them it was renal disease in diffuse |
|
|
Primary or secondary Raynauds?
|
Look for antibodies
Nailfold capillary changes |
|
|
Vasculopathy of systemic sclerosis
|
Widespread obliterative vasculopathy
Concentric proliferation and thickening of intima Fibrosis of adventitia No inflammation Causes raynauds, scleroderma renal crisis, pulmonary artery htn |
|
|
Antibody predicting risk of scleroderma renal crisis
|
Anti- RNA polymerase III
|
|
|
Gastric antral vascular ectasia
|
Watermelon appearance
Scleroderma |
|
|
Specific joint finding in systemic sclerosis
|
Tendon friction rub
Fibrinous deposits on tendons/sheaths |
|
|
Genetics of systemic sclerosis
|
Complex
RR=13 with 1st degree relative |
|
|
Appearance of kid with Kawasaki's disease
|
Well-nourished and acutely ill
Erythematous palms Diffuse rash Red eyes, lips, tongue |
|
|
Kawasaki's disease clinical presentation
|
Fever > 5 days
Bilateral conjunctival injection Oropharynx -- erythema/swelling of lips, fissured, strawberry tongue Extremities -- erythema of palms and soles, induration of hands and feed, desquamation Polymorphous rash Cervical adenopathy (in half) -- typically unilateral |
|
|
Symptom of Kawasaki's in recovery
|
Desquamation from nail bed down
|
|
|
Kawasaki's pathophysiology
|
Systemic vasculitis in middle sized arteries
Predilections for coronary arteries |
|
|
Kawasaki's disease and the heart
|
Coronary artery dilation/aneurysm in 20-25% of untreated
Myocarditis (50%) and pericarditis (20-30%) and CHF in untreated |
|
|
Non-cardiac manifestations of Kawasaki's disease
|
RUQ pain associated with hydrops of the gallbladder (14%)
Vomiting and diarrhea (40%) Elevated LFTs (35%) Arthritis (30-60%) Irritability CSF pleocytosis Cranial nerve palsies (hearing loss) Pneumonitis Transient uveitis |
|
|
Kawasaki's disease epi
|
Disease of children <5
Peak <2 Asian>AA>Caucasian>Hispanic FH increases incidence Geographic time space clusters Late winter-early spring No person to person transmission documented |
|
|
Kawasaki's disease etiology
|
Seems like it should be viral, but no virus IDed
Infection causing immune disease in genetically predisposed host Infection producing superantigen toxin |
|
|
Kawasaki's disease treatment
|
ASA (acetylsalycilic acid)
IVIg ECHO and diagnosis and at 6-8 weeks --treatment of aneurysms if necessary |
|
|
Kawasaki's disease outcomes
|
Self-limited illness
IVIg reduces coronary aneurysms to <5% and mortality from 1-2% to 0.1% |
|
|
Neonatal lupus
|
Transplacental passage of autoantibodies SS-A (RO) and SS-B (La)
Skin lesions Heart block - slow HR, can be irreg Hepatic abnormalities Cytopenias (usually thrombo) |
|
|
Cardiac pathology in neonatal lupus
|
Onset 16-30 weeks gestation
Characteristic lesion ins complete AV block Associated with myocardiopathy, hydrops fetalis 14% mortality at 3 months 20% at 3 years 70-100% require pacemaker by 15 |
|
|
Who has lupus in neonatal lupus?
|
Sometimes no one
Mothers must have antibodies, but don't necessarily have lupus syndrome Some developing it Babies don't have lupus, but are at increased risk of developing autoimmune disease later in life |
|
|
Skin lesions in neonatal lupus
|
6 weeks - 6 months (antibodies cleared)
Raccoon eyes, discoid lesions Frequently photosensitive Persistent telangectasias (20%) Hypopigmentation (20%) |
|
|
Liver disease in neonatal lupus
|
Hepatomegaly and cholestasis
Elevated LFTs Usually resolves spontaneously |
|
|
Neonatal lupus
|
Unknown incidence
Most babies with congenital heart block have neonatal lupus Antibodies are necessary but not sufficient 1-2% of Ro/La positive moms have babies with neonatal lupus, higher rates with siblings of babies with disorder |
|
|
Treating neonatal lupus
|
Sun protection
IVIg/corticosteroids -- heme Fetal heart block may respond to fluorinated corticosteroids Fetal pacemaker if necessary |
|
|
Acute rheumatic fever
|
Inflammatory disease after Strep A
(serotypes M3, M18) Involves heart, joints, CNS, skin, subcutaneous |
|
|
Rheumatic fever epi
|
Kids 6-15
Declining in developed nations (? strains, antibx, hygiene) |
|
|
Rheumatic fever pathogenesis
|
Strep A pharyngitis
Genetically susceptible host (B cell alloantigens, increased mannose biding lectins, certain HLA IIs, etc) Cross reactive antibodies and/or cell mediated Hyperreactive response to strep |
|
|
Erythema marginatum
|
Rash of rheumatic fever
Serpignious border |
|
|
Diagnosis rheumatic fever
|
Major:
Carditis (endo) Migratory polyarthritis Erythema marginatum Subcutaneous nodules Chorea (months later) Minor: fever, arthralgia, ESR/CRP, prolonged PR, previous rheumatic fever/heart disease Preceeding strep: culture, known scarlet fever, elevated ASO |
|
|
Acute rheumatic fever treatment
|
Bed rest
Antibiotics to eradicate and prevent strep pharyngitis Anti-inflammatory meds to suppress inflammation once diagnosis is established |
|
|
What does fracture pattern tell you?
|
Mechanism
Appropriate reduction strategy |
|
|
Simple fracture
|
One line divides the bone into two pieces
|
|
|
Comminuted fracture
|
Multiple fracture lines
Creating more than two piece of bone |
|
|
Open vs closed fracture
|
Closed -- bone remains within skin
Open -- bone is exposed to air -- either because its poked out or because the overlying tissue has been damage -- surgical emergency |
|
|
Treating open fracture
|
Surgical emergency, need to go to OR for irrigation and debridement
In field: sterile dressing, splint, tetanus prophylaxis, IV antibiotics |
|
|
Transverse fracture pattern
|
Failure under tension
|
|
|
Oblique fracture pattern
|
Failure under compression
|
|
|
Spiral fracture pattern
|
Failure due to rotation, twisting
|
|
|
Comminuted with butterfly pattern fracture
|
Failure due to bending
|
|
|
Segmental fracture
|
Two separate fracture lines that create a segment between them
|
|
|
How to describe angulation of fracture
|
Describe where the distal end of the distal piece is
Valgus - lateral, varus- medial |
|
|
How to describe the displacement of fracture
|
% of diameter of bone off from where it should be
cm of shortening |
|
|
Greenstick fracture
|
Young bones
One cortex breaks and the other bends We have to break other side (complete fracture) to treat |
|
|
Buckle or torus fracture
|
Young bones
Usually with compression One cortex wrinkles and the other is normal Very stable Heal quickly Can treat with splint |
|
|
Pathologic fracture
|
Weak bone failing under small stress
Weakening from osteoporosis, cancer |
|
|
Impacted fracture
|
Piece of joint driven down into metaphysis
or End of long bone impaled by shaft |
|
|
What tissue can repair with normal tissue rather than scar?
|
Bone
|
|
|
Callus
|
New bone laid down at site of injury
|
|
|
Phases of bone healing
|
Inflammatory - bone failure and injury of soft tissue and vessels, osteophyte necrosis, hematoma and acute inflammatory reponse
Reparative -- Invasion of pluripotent mesenchymal cells form blood, production of callus (fibrous tissue, cartilage, immature bone), resorption of necrotic bone, crystals of calcium hydroxyapitate, union Remodeling into appropriate mature bone structure |
|
|
Radiographic union vs clinical union
|
Clinical -- callus completely connects
Radiographic -- can no longer see fracture lines |
|
|
Why do hairline fractures appear to get bigger radiographically in first weeks
|
Resorption of necrotic bone tissue in reparative phase
Callus in area in not yet calcified |
|
|
How long does each phase of bone healing take?
|
Inflammatory phase 0-2 week
Reparative phase 2-12 weeks Remodelling 6 weeks - years |
|
|
Treating fractures
|
Reduction:
Traction and then reversal of mechanism open or closed Immobilization cast, traction, external or internal skeletal fixation |
|
|
Colles fracture
|
Fall onto outstretched hand
Dorsal comminution, dorsal angulation, dosral displacement Usually closed reduction Long arm cast (joint above, joint below) Sometimes unstable and need to be pinned |
|
|
Ankle fracture
|
Usually a twisting motion
Reduction is in opposition Displacement requires internal fixation with plates and screws |
|
|
Hip fracture
|
Usually intertrochanteric
Present with shortened, externally rotated leg Usually in elderly Treated with internal fixation |
|
|
Femur fracture
|
Usually in young adults in the setting of many injuries
Standard now is prompt internal fixation Early surgery reduces DVTs and fat emboli Traction may be necessary |
|
|
Why is it important to stabilize fractures in multiple trauma?
|
Makes nursing care, mobilizaiton easier
Thereby improving pulmonary fnc Long bones need to be stabilized to reduce risk of fat embolism and PE |
|
|
What helps bones heal?
|
TGF
Bone morphogenic proteins FGF PDGF IGF-1 |
|
|
TGF in fractures
|
Promotes proliferaton and differentiation of osteoblasts, osteclasts, chondrocytes
Induces synthesis of type II collagen, cartilage proteins Stimulates collagen synthesis in osteoblasts |
|
|
Bone morphogenic proteins
|
Osteoinductive proteins
Chemotaxis and mitosis of mesenchymal cells, differentiation into cartilage, replacement by bone Also regulate hematopoeisis, increase ECM synthesis, influence cell survival |
|
|
BMP2 and BMP7
|
Induce endochondral bone formation in segmental defects
Limited therapeutic approvals |
|
|
FGF in bone healing
|
Proliferation of chondrocytes and osteoblasts
Enhances callus formation |
|
|
PDGF in bone healing
|
Stimulates bone cell growth
Increase type I collagen by increasing osteoblasts Increases osteoclasts |
|
|
IGF and bone healing
|
Stimulates collagen and matrix formation
Stimulates replication of osteoblasts Inhibits collagen breakdown |
|
|
Cytokines and bone resorption
|
Il-1 is most potent
Il-1 and 6 are estrogen suppressed -- mechanism for estrogen protection against bone loss? |
|
|
Thyroid hormones and bone
|
Thyroid hormones stimulates osteoclastic bone resorption
|
|
|
Parathyroid hormone and bone
|
PTH stimulates resorption physiologically
Therapeutic bolus can induce bone formation, fracture healing |
|
|
Glucocorticoids and bone
|
Inhibit calcium resorption in gut
Causing increase in PTH Bone reabsorption |
|
|
Anatomic features that influence fracture heaing
|
Soft tissue injury
Interruption of blood flow Interposition of soft tissue Bone death caused by xrt, burns Smoking -- decreased blood flow |
|
|
Why might you use external fixation in kids?
|
Can't put a screw into a growth plate
|
|
|
Function of intervertebral bodies
|
Shock absorber
Transmitting compressive loads between vertebral bodies |
|
|
Structure of intervertebral disc
|
Cartilanginous endplate
growth plate of vertebral bodies avascular hyaline cartilage in adults Nucleus pulposus gel-like core of disc proteoglycan, water, type II collagen, elastin Annulus fibrosis concentric rings of type I collagen perpedicular organization results in maximum tensile strength |
|
|
Contents of nucleus pulposus
|
Proteoglycans -- aggrecan
allows for water absorption osmotic properties to resist compression Chondrocyte-like cells Type II collagen Elastin |
|
|
Aggrecan
|
Found in nucleus pulposus
Proteoglycan Protein with glycosaminoglycan side chains Aggregates Provides anionic charge that attracts Na+, which attracts H20 Allows for turgor |
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Vascular supply to intervertebral disc
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Mostly by diffusion from vertebral capillaries through endplate cartilage
Some capillaries at outside of annulus Low oxygen tension, high lactic acid in middle of pulposus |
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Innervation of intervertebral disc
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Only periphery of annulus is innervated normally
Nerve ingrowth in degenerated lumbar discs |
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Disc aging
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Dehydration
Disc becomes fibrotic Type I collagen predominates over II Collagen fibrils cross-link Reduced tissue strength and ability to resist compressive loads |
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What causes disc aging?
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Idiopathic loss of blood supply to endplate (loss of nutrients)
Non-enzymatic glycation -- forms advanced glycation end products -- cross linking of proteins -- stickier, brittle disc |
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Disc degeneration
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Disc aging that is happening too fast
Related to Microfracture of endplate Rupture of inner annulus |
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Risk factors for disc degeneration
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Genetic
Smoking Occupation Obesity |
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Degenerative disc disease
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When a degenerated disc causes pain
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Neurovascular changes in degenerative disc
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Ingrowth of capillaries possible 2/2 loss of hydrostatic pressure
New vessels release neurotrophic factors Ingrowth of neurons -- including nociceptive -- pain of DDD |
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Disc herniation
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Pulposus escapes outward
Usually through posterolateral corner of annulus Often in young adults Presents with pain from impingment on lower number root |
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Protrusion of disc
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Pulposus is covered with thin layer of annulus
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Extrusion of disc
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Pulposus ruptures through the full thickness of hte annulus and lies under the posterio longitudinal ligmanet or under dural sac
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Sequestration of disc
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Herniated disc fragment loses contact with original disc
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Most common kind of disc herniation
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Posterolateral
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Far lateral disc herniation
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In lumbar region will cause higher number root impingement (radicular pain)
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Natural history of disc herniation
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Radicular pain
Slow resolution over 6-8 weeks If symptoms persist or there is progressive weakness/numbness discectomy may be appropriate |
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Radicular vs referred leg pain
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Radicular follows dermatome often includes tingling or weakness in root distribution
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Degenerative disc disease
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Lumbosacral jct
Loss in disc height Spinal instabilty --quality of motion, instant axis of rotation Axial back pain referred leg pain Activity related mechanical back pain |
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Instablity catch
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Symptom of DDD
Sharp back pain when bending forward Or getting up from bent forward position |
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Treating disc degeneration
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Less than satisfying
Can fuse segment, replace disc, soft/dynamic stabilization surgically |
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Disc height loss
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Narrowing of space from degeneration
May lead to disc tear Buckling of annulus Overloading of facet joints --secondary degeneration |
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Degenerative spondylisthesis
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Abnormal translation (subluxation) of vertebral body 2/2 degenerative disc change
Subsequent spur formation, subchondral sclerosis, hypertrophy, ossification of ligament, hypertrophic facet arthrosis --trying to stabilize |
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Pro vs retro listhesis
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Pro - forward - lower lumbar spine
Retro - back -- upper lumbar spin |
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Degenerative spondylisthesis epi
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Middle aged or elderly femal
L4-5, L5-S1 |
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Degenerative spondylisthesis symptoms
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Activity related mechanical back pain
Baseline axial back pain Referred or radicular leg pain |
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Treating degenerative spondylisthesis surgically
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When conservative therapy fails to relieve pain
Laminectomy to decompress nerve roots -- improve leg pain Fusion of segment -- improve back pain Don't do a total disc replacement -- might make things more unstable |
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Spinal stenosis
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Symptomatic narrowing of spinal canal
2/2 restabilization process in degenerative disc disease progression Osteophytes around disc margin, facet joints |
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Spinal stenosis epi
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Elderly
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Spinal stenosis symptoms
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Neurogenic claudication
back and leg pain precipitated by standing for a short duration or walking a short distance relieved by bending forward Bicycling rarely causes pain (different than vascular claudication) |
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Treating spinal stenosis
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Conservatively because these are elderly, comorbid pts
---spinal epidural steroid injections Decompression laminectomy |
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Degenerative scoliosis
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Lateral listhesis of degenerated sgement
Wedging of listhetic vertebra lateraly Sharp scoliotic angle Short segment involved Axial back pain, spinal stenosis |
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What is the gross pathology bones?
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X rays
Tumors have different borders, periosteal reaction, calcific patterns |
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XRT appearance of a slow growing bone tumor
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Geographic margin
Solid periosteal rxn |
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Radicular pain 2/2 foramen stenosis
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Higher number nerve in cervical
Lower number elsewhere |
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Cocciodyes osteomyletis appearance on xray
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Permeative lesion
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XRT appearance of an intermediate speed growing tumor
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Ill defined or moth eaten border
Shells periosteal rxn |
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XRT appearance of fast growing bone tumor
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Permeative border
Lamellar periosteal rxn |
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XRT apperence of fastest growing bone tumors
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No apparent border
Absent or spiculated periosteal response |
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What lytic bone lesions always have a geographic border?
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Cysts
Chondroma Fibrous dysplasia Giant cell tumor |
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What lytic bone lesions always have a moth eaten or permeative border?
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Osteosarcoma, Ewing's sarcoma
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What lytic bone lesions can have any kind of border?
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Chondrosarcoma
Ostemyelitis Metastasis |
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Osteoid osteoma
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Benign
Richly vascularized, woven bone and osteoid surrounded by sclerotic rxn Diaphyseal |
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Presentation of osteoid osteoma
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Adolescent
Femur (50%) Progressive pain, esp at night, relieved by aspiriin |
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Osteosarcoma
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Malignant
Aggressive bone forming tumor Often around knee Metaphyseal Hematologic mets -- lungs Osteoblastic, chondroblastic, fibroblastic, telangectatic histologies |
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Osteosarcoma presentation
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10-25 years old (ex paget's)
M 2: 1 F Persistent pain, sometimes with localized swelling, pathologic fracture |
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Treating osteoscarcoma
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Need surgeon, radiation onocology, medical oncologist
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Osteochodroma
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Benign osteocartilaginous exostosis
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Osteochondroma clinical
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Hard diaphyseal mass in adolescence
Grows with long bone |
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Chondroma
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Benign
Usually solitary Often incidental finding Polylobulated cartilaginous nodules w/ bland cytology |
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Ollier's syndrome
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multiple chondromas with fibrous dysplasia
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Mafucci's syndrome
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Multiple chondromas with angiomata
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Chondrosarcoma
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Malignant
Adult with slowly growing mass Cytology of chondrocytes is key to diagnosing |
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Giant cell tumor
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Osteoclastoma
Abdundant multinucleate giant cells, spindle cells Benign but aggressive bone tumor Lytic metaphyseal lesion Usually around knee Recurrent is common if primary surgical treatment is inadequate |
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Giant cell tumor epi
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age 20-40
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Giant cell tumor presentation
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Pain
Pathologic fracture |
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Ewing's tumor
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Meta/diaphyseal permeative lesion with prominent periosteal rxn
Small round cell malignancy of childhood (5-15_ Primitive mononuclear cells |
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Chondrosarcoma
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Malignant
Adult with slowly growing mass Cytology of chondrocytes is key to diagnosing |
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Giant cell tumor
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Osteoclastoma
Abdundant multinucleate giant cells, spindle cells Benign but aggressive bone tumor Lytic metaphyseal lesion Usually around knee Recurrent is common if primary surgical treatment is inadequate |
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Giant cell tumor epi
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age 20-40
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Giant cell tumor presentation
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Pain
Pathologic fracture |
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Ewing's tumor
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Meta/diaphyseal permeative lesion with prominent periosteal rxn
Small round cell malignancy of childhood (5-15_ Primitive mononuclear cells |
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Ewing's sarcoma presentation
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Pain
Metastatic lesions to other bones, lung |
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Ewing's sarcoma genetics
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11:22 translocation
EWS - FLI - 1 fusion protein |
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Fibrous dysplasia of bones
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Fibro-osseus proliferation on long bones, causing deformity
Diaphyseal, intramedullary fusiform lesions w/ ground glass appearance on XRT Can be associated with growth deformities W>M Spindle cells in a collagenous matrix in woven bone |
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Blastic vs lytic lesions
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Blastic -- reactive growth of new bone
Lytic - destruction of existing bone |
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Incidence of flat feet
Club feet |
1/20
1/1000 |
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Incidence of spina bifida
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1/1000
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Incidence of hip instability
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1/100
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Malformation
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Primary structural defect
Error in tissue formation |
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Disruption
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Destruction of previously normal structures
Eg Congenital Banding Syndrome |
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Deformation
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Alteration in shape or position of a normally formed tissue
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Dysplasia
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Structural disorder caused by abnormal tissue differentiation
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If you see multiple minor malformations...
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Look for a major malformation
5 x higher rate |
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Sequence of congenital anomalies
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Multiple anomalies steming from same mechanical problem
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Syndrome of congenital anomalies
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Multiple anomalies that are pathogenically related
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Metatarsus Adductus
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Forefoot adduction with normal hindfoot
Lateral border of foot is curvilinear Resolves w/o treatment, casting/stretching if severe 1/1000 |
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Clubfoot
Talipes equinovarus |
Forefoot adduction, hindfoot varus and equinus
1/1000, M>F Treat with manipulation and serial casting Surgery if unsuccessful |
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Rotational deformity of lower limb
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Toeing in and out
Common Often outgrown without incident May arise from foot, femur, tibia |
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Genu varus and valgus
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Varus until 18 months
Neutral Valgus 3-4 years Resolution to slight valgus in adulthood |
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Genu varus/valgus and disease
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Pathologic process may exaggerate normal curvature
Concerning -- assymetric, late/persistent, functional impairment or pain |
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Development dysplasia of the hip
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Disolocatable, subluxable, shallow (dysplastic) acetabulum
1/100 in US Females 8 : 1 males |
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Normal development of hip joint
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Rounding of the femoral head
Depth of acetabulum Depend on maintained articulation |
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Risk factors for hip dysplasia
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First born
Femal Breech Family history |
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Etiology of developmental hip dysplasia
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Ligamentous laxity
Excessive femoral anteversion Intrauterine malposition Genetic factors |
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Associated findings with developmental hip dysplasia
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Torticollis
pes calcaneovalgus metatarsus adductus plagiocephaly extension contractures of the knee |
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Physical findings in developmental hip dysplasia
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Can dislocate or relocate
Limited hip abduction, rotation Assymetric gluteal folds Limb length discrepancy Lordosis, waddling gate |
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Barlow maneuver
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Dislocating a dysplastic hip
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Ortalani maneuver
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Relocating a dysplatic hip
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Imaging in developmental hip dysplasia
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Ultrasound of developing femoral head
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Treating developmental hip dysplasia
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Earlier is better
Pavlik harness to hold femoral head in acetabulum Closed/open reduction and spica cast |
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If untreated, developmental hip dysplasia
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Pain
Degenerative arthritis Limb length discrepancy Waddling gait -- weight thrown over down leg |
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Scoliosis
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Lateral and rotation deformity of spine
Outward rotation at convexity Inward at concavity |
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Scoliosis etiology
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Congenital -- vertebral anomaly (often associated with others)
Idiopathic (although hereditary) Neuromuscular |
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Idiopathic scoliosis epi
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Female 7 : 1 Male
2/1000 > 20 degrees 1/9000 require surgery |
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Exam in scoliosis
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Not painful
Bend forward and see rib prominence |
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Treating idiopathic scoliosis
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Observation
Bracing -- reduces progression Spinal fusion |
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Risk factors for progression of scoliosis
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Female
Young onset Delayed maturity Large curve |
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Consquences of untreated scoliosis
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Low self-esteem
Psychosocial impact ?cardiopulmonary compromise Pain |
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Limping child DDx
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Spine, knee, hips, feet
If painful: trauma, infection, inflammation, tumor If painless: structural musculoskeletal, neurologic, metabolic |
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Legg-Calves-Perves disease
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Avascular necrosis of femoral head
Regeneration Self limited in 24-36 months |
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Legg-Calves-Perves presentation
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3-12 years (avg 6)
Boys 5 : 1 girls Limping, muscle spasm, decrease ROM, sometimes pain |
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What results in long term problems Legg-Calves-Perves syndrome
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Femoral head not remodelling to a smooth surface
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Risk factors for bad long term problems after Legg-Calves-Perves syndrome
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Female
Onset > 10 Loss of motion Femoral head deformity |
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Treating Legg-Cavles-Perves
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NSAIDs
Maintain motion Braces Surgery |
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Slipped capital femoral epiphysis
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Displacement of femoral neck on proximal femoral epiphysis
Presents as hip pain or referred knee pain Early adolsecents Obese (more than 50% are in 95%) Surgical stabilization with pinning 2/100000 |
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Septic arthritis of hip presentation
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Fever
Limp Refusal to walk Hip held in flexion, external rotation ROM is extremely painful |
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Septic arthritis of hip diagnosis
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Fluid on us
Joint wide on xrt Increased WBC, CRP, ESR Need to aspirate to be sure |
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Septic arthritis of hip treatmetn
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Urgent surgical debridement to avoid destroying joint
Antibiotics |
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VACTERL
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Vertebral abnormalities
Anal atresia CV abnormalities Tracheoesophageal fistula Esophageal atresia Renal or radial anomalies Limb abnormalities -- preaxial |
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Klippel-feil syndrome
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Webbed neck
Low hairline Congenital fusion of C spine |
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What is early finding in hip problems?
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Loss of internal rotation
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Flexible flat feet
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1/7 people
Usually not a problem |
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Tarsal coalition
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Rigid flat feet
Abnormal connection between tarsal bones Starts as fibrocartilagenous and ossifies Not all are symptomatic If so, usually starts around 8-12 yrs Calcaneonavicular -- common Talo-calcaneal -- more severe |
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Tarsal coaltion problems
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Loss of subtalar motion
Recurrent ankle sprains Cannot stand on outside of foot |
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Bount's disease
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Progressive genu varus
Changes to medial proximal tibial physis Seen in early walkers, obese May require bracing |
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Genetic rickets
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X linked disorder in reabsorbing phosphate in kidney
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What does a dysplastic hip look like on xray
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If dissolcated - high riding, posterior dislocated
Slow to ossifiy |
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Galleazzi sign
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Late finding in developmental hip dysplasia
Shorter leg on effected side Missed diagnosis |
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Indications for scoliosis surgery
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Posterior spinal fusion
More than 50 deg curve in kids More than 60 in adults |
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Patients who get LC Perthes disease
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Delayed bone age
Short stature ?ADHD association |
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Slipped capital femoral epiphysis epi
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2 /100K worldwide
Higher in polynesian, black kids Slightly more in boys 25-30% bilateral |
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Slipped capital femoral epiphysis exam
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Pain on internal rotation
If unable to bear weight, instability, AVN, degenerative joint risk |
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Soft tissue swelling on xrt
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Indicates site involved
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RA on xray
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Symmetric soft tissue swelling centered at joints
Demineralization Uniform joint space loss Erosions at joint margins Subluxation Small joints, rarely DIP |
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Psoriatic arthritis on xray
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Fusiform soft tissue swelling (sausage digit)
Increased bone density Erosions at joint margin Ankylosis late |
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Gout on xrt
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Eccentric soft tissue masses
Preserved joint space Sclerotic erosions with overhanging bone lip, can be away form joint |
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Osteoarthritis on xrt
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Asymmetric soft tissue swelling
Non-uniform joint space narrowing Osteophytes --usually marginal |
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Uniform joint space narrowing
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Inflammatory process
RA, septic, seronegatives |
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Subluxation in hand
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With erosions -- RA
Without erosions - SLE |
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Osteophytes
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Bone formed by abnormal cartilage
See in degenerative arthritis Extension of articular surface |
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Periostitis
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Seronegative spondyloarthropathies
Inflammatory response by periosteum Formation of new bone |
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Anklyosis
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Fusion of bone across joint space
Late manifestation of SN spondyloarthropathies, septic, osteoarthritis Not seen in RA except in carpals/tarsals |
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Subcondral bone growth
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Typical of OA
Reflects regenerating bone Eburnation (ivory-like) on xrt |
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Syndesmophytes
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Ossification of annulus
Parallel to anterior cortex of vertebral bodies Characteristic of ank spond |
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Chondrocalcinosis
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Calcification of cartilage
Typical of pyrophosphate crystal deposition disease Also can be seen in post trauma, OA |
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Calcification of tendons, soft tissue
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Hydroxyapatite
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Calcinosis
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Mass like calcification
In perarticular soft tissues Scleroderma, dermatomyositis |
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What kind of bone growth in seronegatives?
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Periosteal
Ankylosis |
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Genetic pleiotropy
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Genes that have more than one discernible effect in body
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Genetic (locus) heterogenetity
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Different genes resulting in the same disease phenotype
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Osteogenesis imperfecta
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Inherited defect in type I collagen
Resulting in bones that fracture easily |
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Why so many different types of osteogenesis imperfecta?
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Different mutations
Multiple steps in collagen synthesis (two different pro-collagen types, assembly, etc) |
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Worst type of osteogenesis imperfecta?
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Type II
Perinatal lethal Usually novel AD mutations Minimal calvarial calcification, in utero fractures |
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Type I osteogenesis imperfecta
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Mild type
Fractures with walking in childhood, increase again in old age Normal stature Blue sclera Conductive hearing loss in 50% of families IA - normal teeth IB - dentiogenesis imperfecta Easy bruising (thin skin) Hypermobility Aortic root dilation MV prolapse |
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Type I osteogenesis imperfecta histology
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Thinner but normal lamella
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Type IV osteogenesis imperfecta
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AD
Mild/mod bone problems generalized osteoporosis, fracures, curved limbs Slightly short stature Dentinogenesis common Hearing problems variable |
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Type III osteogenesis imperfecta
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AD (AR)
Progressively deforming bones Some deformity at birth Very short stature Dentinogenesis common Hearing loss common Scleral vary Long bones are short and curved |
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Genes for collagen I
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Chromsomes 7 and 17
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Dominant negative effect
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Production of misfolded protein aggregates and depletes associated proteins
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DDx kids with multiple fractures
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Child abuse
Osteogenesis imperfecta Vitamin deficiency |
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Diagnosing osteogenesis imperfecta
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Genetic analysis
Dermal fibroblast culture assay |
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What does collagen do for bone?
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Provides resilience to deformity
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Do bisphosphonates work in osteogenesis imperfecta
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They do increase bone density
This probably does not decrease fracture rate signficantly |
