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171 Cards in this Set

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alloantibodies

=isoantibodies


antibodies produced by one individual that react with antigens from another individual of the same species

central tolerance mechanism

negative selection of T-cells occurs in the thymus when T-cells that react with self antigens are deleted



B-cells in the bone marrow that react with self antigen either undergo receptor editing or apoptosis

AIRE protein

stimulates the expression of peripheral antigens within the thymus so that T-cells develop tolerance

peripheral tolerance 3 mechanisms

T regulatory cells


activation induced cell death


anergy

T regulatory cells

self reactive T-cells that suppress antigen presenting cells presenting self antigen via IL-2R and FoxP3

activation induced cell death

either via the mitochondrial/Bcl2 pathway or Fas death receptor pathway

anergy

2 signals are required for full activation of lymphocytes:


antigen couple to self MHC


costimulatory CD28 on the T-cell and B7 on the APC



-absence of the second signal or an inhibitory T-cell costimulatory molecule CTLA4--> anergy

autoimmune thrombocytopenic purpura:


type, antigen, mechanism, manifestations

type: II


antigen: platelet membrane proteins


mechanism: opsonization and phagocytosis of platelets


manifestations: bleeding

pemphigus vulgaris:


type, antigen, mechanism, manifestations

type: II


antigen: intercellular junction proteins


mechanism: Ab activation of proteases--> destruction of the intercellular adhesions


manifestation: skin vesicles

ANCA vasculitis:


type, antigen, mechanism, manifestations

type: II


antigen: neutrophil granule proteins


mechanism: neutrophil degranulation--> inflammation


manifestations: vasculitis

goodpasture syndrome:


type, antigen, mechanism, manifestations

type: II


antigen: non-collagenous basement membrane proteins


mechanism: complement/Fc receptor mediated


inflammation


manifestations: nephritis, lung hemorrhage

insulin-resistant diabetes:


type, antigen, mechanism, manifestations

type: II


antigen: insulin receptor


mechanism: inhibition of insulin binding


manifestations: hyperglycemia, ketoacidosis


pernicious anemia:


type, antigen, mechanism, manifestations

type: II


antigen: intrinsic factor of gastric parietal cells


mechanism: decreased absorption of B12


manifestations: abnormal erythropoiesis/anemia

systemic lupus erythematosus:


type, antigen, manifestations

type: III


antigen: nuclear antigens


manifestations: nephritis, skin lesions, arthritis,


others



poststreptococcal glumerulonephritis:


type, antigen, manifestations

type: III


antigen: streptococcal wall antigens


manifestations: nephritis

polyarteritis nodosa:


type, antigen, manifestations

type: III


antigen: sometimes Hep B antigens


manifestations: systemic vasculitis


reactive arthritis:


type, antigen, manifestations

type:III


antigen: bacterial antigens


manifestations: acute arthritis

serum sickness:


type, antigen, manifestations

type: III


antigen: foreign proteins


manifestations: arthritis, vasculitis, nephritis

type I diabetes:


type, antigen, manifestations

type: IV


antigen: pancreatic beta cells


manifestations: loss of insulin production

multiple sclerosis:


type, antigen, manifestations

type: IV


antigen: CNS myelin proteins


manifestations: demyelination of CNS w/ perivascular inflammation paralysis and ocular lesions

crohn's disease:


type, antigen, manifestations

type: IV


antigen: possibly commensal bacteria


manifestations: chronic inflammation and intestinal obstruction

Guillain Barre:


type, antigen, manifestations

type: IV


antigen: peripheral nerve myelin


manifestations: neuritis, paralysis

immune privileged sites

-testes, brain, eye have hidden antigens


-trauma may expose them-->autoimmune rxn

clinical features of Rheumatoid arthritis

-small joint pain, swelling, and morning stiffness


-pain lasts >1hr, improves w/ movement, worse w/ inactivity


-fatigue/weight loss


-rheumatoid nodules


-pleuritis/pericarditis


-sjogren's syndrome


-interstitial lung disease


-Eye inflammation


-other rare complications (vasculitis, splenomegaly/leukopenia, amyloidosis)

lab tests for rheumatoid arthritis

-rheumatoid factor: sensitive but not specific


-Anticitrullinated protein Ab's: 60-75% of RA patients-->specific not sensitive


-high ESR and CRP


-anemia


-high ferritin


-mildly low albumin

rheumatoid arthritis imaging studies

-soft tissue swelling on X-ray


-late RA may have marginal erosions, and joint space narrowing


-MRI/ultrasound show synovitis

complications of rheumatoid arthritis

-progressive disease in 10% of cases


-permanent joint damage especially in seropositive cases: ulnar deviation, subluxation, atlanto-axial instability


-increased mortality

treatment of Rheumatoid arthritis

DMARDS: eg. methotrexate


Biologics: anti-TNF eg. infliximab


Corticosteroids: rapid control


NSAIDS: non-disease modifying for pain relief

Juvenile Idiopathic Arthritis: systemic onset



(Still's disease)

-fever 1-2 times/day


-salmon colored, evanescent rash


-polyarthritis in ~50% of cases


-pericarditis in 30% of cases


-macrophage activation syndrome: leukopenia, anemia, ALT/AST, very high ferritin


-treat w/ IL-1 blocker

Juvenile Idiopathic Arthritis: oligoarticular

-most common form of JIA


-onset before 5 yrs


-arthritis in 1-4 joints


-uveitis


-ANA+ in 40-80% --> higher risk of uveitis


-treat w/ NSAIDS, steroids, methotrexate

Juvenile Idiopathic Arthritis: polyarticular

can be RF +/-


RF- affects mostly larger joints


RF+ affects smaller joints, starts after 12 yrs, may persist into adulthood


-treat w/ DMARDS, biologics


Juvenile Idiopathic Arthritis general features

-high rate of uveitis


-likely to resolve before adulthood


-dysfunction rather than pain


-micrognathia


-accelerated bone growth

sjogren's syndrome: 'sicca' or glandular features

-dry eyes


-dry mouth


-dry nose/upper airway


-dry skin/vagina

extraglandular features of sjogren's

-arthralgias/arthritis


-raynaud's


-interstitial nephritis


-liver involvement


-neuropathy


-vasculitis


-LYMPHOMA

diagnosis of sjogren's

-SSA (Ro), SSB (La) Ab's


-schirmer test for tears


-eye stains


-saliva measurements


-salivary gland biopsy

sjogren's treatment

-symptomatic management w/ lubricants, cholinergic agents etc


-mild extraglandular: hydroxychloroquine, low dose steroids


-severe extraglandular: high dose steroids, DMARDS, biologics

genetic component of lupus

-HLA loci and possibly complement


-high monozygotic concordance


-20x relative risk in close relatives

epidemiology of lupus

-1/2000 prevalence


-8:1 female: male


-more common in african americans

clinical features of Lupus

-photosensitivity


-malar rash


-discoid rash


-oral ulcers


-arthritis


-serositis


-renal involvement


-neurologic: psyochosis


-hemolysis/cytopenia


-immunologic rise in specific Ab's

ANA for lupus

-used as a screening test (sensitive not specific)


-high titer >160:1 is common in lupus and other autoimmune disorders


-low + titers are common in healthy ppl

Lupus specific antibody tests

dsDNA- pathogenic--> nephritis



Smith- may predispose to GI involvement

limited systemic sclerosis


'CREST syndrome'

-skin involvement distal to elbows and knees


-raynaud's phenomenom


-pulmonary hypertension


-pulmonary fibrosis


-anti-centromere antibodies+


-dilated capillary loops in the nailfolds

diffuse systemic sclerosis

-total skin involvement


-raynaud's phenomenon


-sometimes anti-topoisomerase antibodies+


-capillary dilation and destruction in nailfolds


-early organ involvement

internal organ involvement in systemic sclerosis

-esophageal and GI dysmotility


-GERD


-pulmonary HTN and fibrosis


-renovascular HTN and progressive renal insufficiency


-myositis/arthritis

pathogenesis of systemic sclerosis

-autoantibodies and dysregulated lymphocytes


-vascular abnormalities


-fibrosis via myofibroblasts

examples of localized vs systemic autoimmune diseases

localized: hashimoto thyroiditis, autoimmune atrophic gastritis, autoimmune hemolytic anemia



systemic: SLE, sjogren's, systemic sclerosis, RA

4 ANA immunofluorescence patterns

-homogenous: non-specific


-speckled: non-specific, includes Ro, La, smith + others


-nucleolar: systemic sclerosis


-centromere: CREST syndrome

sjogren's pathology

-lymphocytic (mostly CD4+) infiltrate of lacrimal and salivary glands-->fibrosis


-may also progress to involve synovia, lungs, peripheral nerves

systemic sclerosis pathology

-extensive fibrosis


-sclerotic atrophy of the skin


-GI fibrosis w/ villi loss and barretts esophagus


-renovascular fibrosis-->HTN-->kidney failure


-pulmonary fibrosis and HTN


-pericarditis, myocardial fibrosis

rheumatoid arthritis pathology

CD4+ cells, plasma cells, macrophages in synovium--> hyperplasia, edema, fibrin deposition, increased vascularity--> pannus formation--> joint deformity and fusion

rheumatoid factor

-forms immune complexes


-underlies most extra-articular manifestations


-not always present or specific

Hydroxychloroquine mechanism and use

-normally an anti-malarial


-accumulates in lysosome often of B-cells


-used for Lupus or RA in combination w/ other drugs

side effects of hydroxychloroquine

-accumulation in retina causing damage that requires monitoring

sulfasalazine mechanism and uses

-NFkB signal inhibition


-causes T-cell apoptosis


-used in combo for RA and spondyloarthropathies

sulfasalazine side effects

hemolytic anemia in G-6-PD deficiency

methotrexate mechanism and uses

-fist line therapy alone or in combo for rheumatoid disease


-folate analog that inhibits thymidine synthase and blocks purine synthesis


-antineoplastic at high doses


methotrexate side effects

-mucositis


-liver damage


-teratogen/abortifacient


-renal clearance via OATP

azathioprine mechanism and use

-prodrug that inhibits purine synthesis--> B and T cell death


-used in SLE, vasculitis, myositis

azathioprine side effects

-bone marrow suppression


-infection


-malignancy


-metabolized by TPMT

leflunomide mechanism and use

-prodrug with long half life due to enterohepatic circulation


-blocks de novo pyrimidine synthesis


-used for arthritis and vasculitis

leflunomide side effects

-diarrhea


-high ALT/AST


cyclophosphamide mechanism and use

-prodrug activated by CYP


-DNA alkylating agent


-suppresses T-cells


-used in combo for vasculitis


-used in chemotherapy

cyclophosphamide side effects

-bone marrow suppression


-infertility


-alopecia

Infliximab mechanism and use
-chimeric Ab against TNFa
- used for arthritis, ankylosing spondylitis
Side effects of infliximab
-infusion rxn
-infections esp. TB
-worsens heart failure
-development of Ab-->tolerance
Abatacept mechanism and use
-fusion protein: IgG and CTLA-4
-binds B7 and blocks CD28
-used in RA
Side effects of abatacept
-Infusion rxn
-infection
-immunization antagonism
Rituximab mechanism and use

-chimeric Ab that binds CD20 on B cells
-used in RA after failure of TNF drugs

Signs of vasculitis

-prolonged constitutional illness
-multi organ involvement
-recurrent URIs
-pulmonary renal syndrome
-palpable purpura
-eye inflammation
-peripheral neuropathy
-new headache in elderly

Wegener's granulomatous
(GPA)

-C-ANCA/PR-3 positive
-often presents w/ sinonasal disease
-pulmonary: nodules, alveolitis/hemorrhage
-pauci-immune glomerulonephritis
-neuropathy
-arhtralgias/malaise/fatigue
-skin purpura, nodules, oral ulcers, gangrene
-cardiac disease
-GI

Churg Strauss syndrome
(EGPA)

-3 main features: eosinophilia, extra vascular granulomas, necrotizing vasculitis
-usually begins w/ severe asthma and upper airways symptoms-->high eosinophilia and lung infiltrates-->vasculitic phase often w/o glomerulonephritis

takayasu's arteritis

-large vessel involvement (1' aortic branches)


-females <40yrs mostly


-arterial stenosis--> fatigue, weak pulses

giant cell arteritis

-most common vasculitis


-mostly affects temporal artery--> jaw claudication, headache, vision loss


-occurs mostly in the elderly


-abnormal artery on biopsy


-often comes w/ polymyalgia rheumatica

polymyalgia rheumatica

-severe arthralgia, stiffness and weakness


-often in the hips and shoulders


-worse in the morning


-must occur after 50 y/o


-must be monitored for giant cell arteritis


kawasaki's disease

-exclusively pediatric vasculitis


-affects primarily the coronaries-->aneurysms

pathogenesis of osteoarthritis

repeated stress on the avascular articular cartilage--> release of chemo/cyto-kines that stimulate chondrocytes to grow and secrete matrix degrading enzymes--> focal cartilage loss, osteophyte formation, synovial inflammation, cyst formation in the sclerotic subchondral bone

clinical features of osteoarthritis

-pain w/ usage of joint relieved w/ rest


-insidious onset


-short morning stiffness


-joint instability


-late stage disease may cause pain at night/rest


-on exam: joint line tenderness, effusions, bony enlargements, laxity of the knees

therapy fo osteoarhtritis

-acetaminophen is first


-NSAIDs next


-intraarticular steroids


-alternative supplements: hyaluronic acid, glucosamine


-muscle strengthening to stabilize joint


-assistive devices


-weight loss may help


-eventually total joint replacement

pathogenesis of septic arthritis

-hematogenous seeding of joints


-often by gram + bacteria


-synovial tissue has no basement membrane to prevent bacterial access


diagnosis of septic arthritis

-acute onset monoarthritis often in the knee


-fever, high WBCs, other extraarticular signs of infections


-synovial fluid opaque, WBCs above 50k and PMNs over 90%


-culture fluid

complications and treatment of septic arthritis

-osteomyelitis, joint destruction, ankylosis


-treat w/ frequent drainage and IV antibiotics

disseminated gonococcal infection

-causes either migratory arthritis and tenosynovitis or monoarthritis


-may also pustular rash


-difficult to culture from joint but may be present in other places


-rapid response to antibiotics

parvovirus B19 infection

-children likely to have slapped cheek rash


-adults likely to get polyarticular arthritis similair to RA


-diagnose via IgM


-often resolves spontaneously


-treat w/ NSAIDs

Hep C arthritis

-occurs in 20% of cases


-half of cases have +RF


-prone to develop cryoglobulinemic vasculitis


-antiviral therapy

Hep B arthritis

-acute onset


-symmetric small joint polyarthritis/tenosynovitis


-associated w/ fever, urticaria/maculopapular rash


-early finding in hep B


-predisposed to polyarteritis nodose

rubella arthritis

-migratory polyarthritis in 30% of cases


-can also occur w/ vaccination


-may precede rash


-NSAIDs


tuberculous arthritis

-chronic monoarthritis of large joint


-AFB not always +


-multi-drug combo effective

fungal arthritis

-usually chronic monoarthritis


-balstomycosis cause an acute septic joint


-consider immune status and geographic location

acute rheumatic fever

-cross reactive Abs against strep


-Jones criteria: Joints, cardiO, Nodules, Erythema marginatum, Sydenhams chorea


-migratory polyarthritis


-aschoff bodies in the heart


-treat w/ penicillin, NSAIDs, possibly prednisone

lyme disease

-borrelia burgdoferi transmitted by ixodes scapularis nymph


-early symptoms: erythema migrans, migratory polyarthralgias


-disseminated symptoms: meningitis, neuropathy, heart block etc


-persistant: encephalitis, inflammatory monoarthritis


-diagnosed by elisa and western blot


-treat w/ doxycycline

gout pathogenesis

-urate concentration in the serum>6.8


-monosodium urate crystals form and deposit in the joints


-mostly underexcretion not over production/intake


-risks include hypertension, obesity, CVD, renal insufficiency, alcohol/BEER, high fructose corn syrup, red meat, fish, family history

Gout presentation

-episodic joint pain, swelling, redness


-usually starts as monoarticular in big toe


-affects medium and and small joints

gout diagnosis

joint aspiration will show high leukocyte count 50-100K and negatively birefringent monosodium urate crystals


-serum uric acid is not helpful in acute attacks


-tophi may be seen in advanced cases


-eventually kidney stones may develop

probenecid

-increases renal clearance of uric acid by blocking proximal tubular reabsorption


-may increase risk of kidney stones


-ineffective with kidney disease


-blocks OATP-->drug interactions

allopurinol

-inhibits xanthine oxidase


-should be used in combo with colchicine


-acute lowering of uric acid may precipitate gout flair


-goal to decrease uric acid <6.0


-may cause hypersensitivity can be severe


-may have significant interactions w/ drugs affecting purine synthesis

rasburicase

-uric acid degrader


-not commonly used except in pediatric patients with tumor lysis syndrome

colchicine

-suppresses inflammatory response to crystals


-used in acute attack and prophylactically


-commonly causes diarrhea and other GI effects

Pseudogout

-calcium pyrophosphate dihydrate crystal deposition disease


-presents similarly to gout but may also be associated with osteoarhtritis


-often seen in wrists, knees, shoulders and hips


-short rhomboid, positively birefringent crystals


-risk w/ increasing age


-anti-inflammatory therapy, no specific treatment

pattern of osteoarthritis

heberden (DIP), bouchard (PIP)


wrist (CMC)


bunions


Hips


knees


neck


lumbar spine

pattern of rheumatoid arhritis

symmetric


PIP/MCP


wrist (CMC)


elbow


shoulder


knee


less often the neck

pattern of Ankylosing spondylitis

affects the lumbar spine and sometimes the neck as well

pattern of psoriatic arhritis

DIP


neck


lumbar spine

pattern of reactive arthritis

usually in the ankles and lumbar spine


can affect the knee

pattern of gout

toes (esp. big toe)


ankles


knees

pattern of tendonitis

common at the shoulder


elbow


knee


ankle

pattern of bursitis

elbows


shoulders


hips


knees

common causes of acute monoarthritis

septic joint


gout


pseudogout


fracture


hemarthosis

common causes of chronic monoarthritis

mechanical trauma


psoriatic arthritis


rheumatoid arthritis


TB


neuropathic pain

common causes of distal symmetric joint pain

rheumatoid arhtritis


lupus


psoriatic arthritis


gout/pseudogout

common causes of DIP pain

heberden osteoarthritis


psoriatic arthritis


gout

common causes of oligoarthritis (<4 joints)

psoriatic arthritis


reactive arthritis


lyme


gout


pseudogout


rheumatoid arthritis

common causes of spondylitis/sacroiliitis

ankylosing spondylitis


reactive arthritis


psoriatic arthritis


IBS


JIA

common causes of enthesopathy

reactive arthritis


ankylosing spondylitis


psoriatic arthritis


IBS

common causes of arthritis + rash

lupus


vasculitic urticaria


dermatomyositis


psoriatic arthritis


reactive arthritis


sarcoidosis

common causes of polyarthralgia (>4 joints)

fibromyalgia


hypothyroidism


viremia


osteomalacia

Normal synovial fluid analysis

viscous, clear


WBC: 0-150


%PMN: 0-50


no crystals


culture negative

Class I synovial fluid

clear, very viscous


WBC: 0-2k


%PMN: 0-50


no crystals


culture negative

Class II synovial fluid

turbid, less viscous


WBC: 2k-50k


%PMN: 50-80


no crystals


culture negative

Class II-III synovial fluid

turbid, less viscous


WBC: 2k-100k


%PMN: 50-90


MSU or CPPD


culture negative

Class III synovial fluid

opaque, not viscous


WBC: >50k


%PMN: >90


no crystals


culture positive

mechanism of acetaminophen

-low potency COX inhibitor


-may have greater effect in CNS vs periphery

use of acetaminophen

-mild-moderate pain


-non inflammatory


-combo with narcotics


-preferred analgesic/antipyretic in children


-initial treatment for osteoarhtritis

side effects of acetaminophen

-safer than most NSAIDs


-hepatic centrilobular necrosis occurs w/ overdose: caused by minor metabolite which depletes glutathione but can be treated with N-acetylcysteine


-analgesic nephropathy w/ chronic high dose uage

side effects of NSAIDs

-inhibition of platelets


-increased risk of MI


-GI bleed/ulcer


-can cause increased Na/H20 retention


-aspirin intolerance syndrome


-delays labor

back pain in pregnancy

-most severe @ 36 wks


-due to relaxin-->sacroiliac hypermobility if in 1st trimester


-due to hyperlordosis in 3rd trimester


-nocturnal back pain may be caused by IVC compression

herniated discs

- most commonly at L4-5 or L5-S1


-pain is worse when sitting and can be:


referred: sclerotomal/aching


radicular: dermatomal/burning/sharp

cauda equina syndrome

-urinary retention


-loss of rectal tone


-saddle anesthesia w/ bilateral leg pain


-caused by large central herniated disc compressing sacral roots

vertebral fracture

-sudden severe back pain w/ any movement


-tenderness over affected level


-usually secondary to osteoporosis

septic spondylitis/sacroiliitis

-progressive pain even w/o movement


-assc w/ fever, chills, infections source


-tenderness over affected level


-staph is common cause w/ IVDU, DM, age risk factors

muscle spasm

-commonly a secondary phenomenon


-can't fully extend


-relief w/ lying down

causes of acute lower back pain

muscle spasm


compression fracture


infection


herniated disk

causes of chronic lower back pain

spondylosis


spinal stenosis


spondyoarthritis


tumor


fibromyalgia

lumbar spondylosis

-pain worse with activity/end of day


-lower back or lateral hip


-relieved w/ lying down


-pressure on the facet joint--> osteoarthritis that can lead to spondylolisthesis, scoliosis, spinal stenosis

spinal stenosis

-caused by a combo of degenerative disk disease and joint disease


-pain is relieved w/ sitting or bending forward


-neurogenic claudication

spondyloarthritis

-associated w/ seronegative spondyloarthritis eg. ankylosing spondylitis, psoriatic arthritis etc


-also seen in JIA


-pain is worse in the morning for >1hr


-relieved with exercise


-starts in sacroiliac and ascends

visceral lower back pain

GI: w/ bowel movements, colitis, ieitis, tumors


GU: menstrual, kidney, prostate, uterus, ovaries


Aortic aneurysm

sacroiliac pain

-pain in back, buttock, thigh


-worse w/ walking and esp. stairs


-positive FABER test

imaging for lower back pain

-low yield of unexpected findings


-MRI has a high false positive rate


-MRI is best but done after plain films


-CT gives good bone resolution

epidemiology of ankylosing spondylitis

-typically occurs in younger men


-similar prevalence to RA (~1%)


-high association w/ HLA B27

clinical features of ankylosing spondylitis

-inflammatory back pain mostly w/ osteoproliferation


-peripheral arthritis: mono or oligo often in lower limbs


-enthesitis


-anterior uveitis


-restriction of chest motion

exam features of ankylosing spondylitis

-pain and stiffness


-measure tragus to wall


- modified schober (lumbar flexion test)


-cervical rotation


- chest expansion


-intermalleolar distance

treatment of ankylosing spondylitis

-physical therapy


- NSAIDs


-anti TNF-a

psoriatic arthritis

-psoriasis usually comes first but not always


-small joints like RA


-asymmetric oligoarticular


-affects DIP joints


-telescoping digits/pencil in cup deformity


-nail pitting


-enthesitis


-axial disease


-dactylitis (sausage finger)


-treat w/ methotrexate and anti-TNF-a

reactive arthritis

-aseptic arthritis 4wks after a GU or GI infection


-organisms include: chlamydia, shigella, salmonella, yersinia, campylobacter, c. diff.


-conjunctivitis


-urethritis


-nail changes, stomatitis, nail changes, balanitis, keratoderma blenorrhagicum


-treat w/ NSAIDs then sulfasalazine


-possibly use antibiotics in chronic case w/ chlamydia cause

IBD related spondyloarthropathies

-asymmetric oligoarthritis in lower extremities


-can be acute/remitting or chronic/frequent


-may see erythema nodosum or pyoderma gangrenosum


-treat w/ NSAIDs, steroids, sulfasalazine and anti-TNF-a (not etanercept)

De Quervain's tenosynovitis

-tendons get trapped in the extensor retinaculum


-caused by repetitive activity using the thumb and radial wrist


-pain over the radial styloid


-worse when make a fist w/ thumb inside and ulnar deviate (finkelstein test)


-splint, steroids, NSAIDs, PT

Carpel Tunnel syndrome

-compression of the median nerve in the wrist


-causes paresthesia and numbness of 1st-3rd digits and radial side of the 4th


-On physical exam: symptoms w/ 1 of 90 degree flexion (Phalens) or extension and tapping (Tinels), thenar atrophy may also be seen


-stepwise treatment: splint, steroids, surgery



trigger finger

-locking of the digit in flexion


-thickened flexor tendon 'pops' past hypertrophied A1 pulley


-corticosteroids or surgery

olecranon bursitis

4 causes:


infection, trauma, gout, arthritis


-distinguished for joint swelling by extension/supination/pronation without pain

lateral epicondylitis

-'tennis elbow'


-pain over extensor tendons reproduced w/ wrist/finger extension, or supination against resistance


-rest, splint/brace, PT*, analgesics, steroids, botox

differential diagnosis of shoulder pain (6)

rotator cuff tendonitis*


acromioclavicular arthritis


adhesive capsulitis


referred from C-spine


glenohumeral arthritis


bicipital tendonitis

rotator cuff tendonitis

-associated w/ repetitive overhead motion


-supraspinatous: pain w/ resisted abduction+internal rotation (empty can, bra strap)*


-infraspinatous: pain w/ resisted external rotation


-subscapularis: pain w/ resisted internal rotation



-severe tears may allow deltoid to pull humeral head upwards-->glenohumeral osteoarthritis


subacromial bursitis

-often occurs w/ rotator cuff injury


-full passive range of motion and no crepitus


-treat w/ aspiration and steroid injection

adhesive capsulitis

-pain and tenderness lateral to coracoid


-limited active and passive range of motion in all directions


-may be caused by DM or imobility


-treat w/ PT, NSAIDs, steroid

bicipital tendonitis

-tenderness over bicipital groove


-pain on supination against resistance


-surgery in athletes and younger patients

radicular neck pain

-test range of neck motion


-extension+ipsilateral rotation+downward pressure on head should reproduce pain (spurling's maneuver)

trochanteric bursitis

-lateral thigh pain and tenderness


-worse w/ climbing stairs or standing up (abduction and external rotation)


-associated w/ leg length discrepancy, IT band tightness, gluteal weakness, back arthritis

pes anserine bursitis

-tendonitis at the attachment just medial and distal to the tibial tuberosity

plantar fasciitis

-tightening/fibrosis of plantar aponeurosis


-usually caused by microtrauma


-rest, NSAIDs, shoe inserts, stretching, possible steroids

Dermatomyositis presentation

-progressive proximal muscle weakness


-symmetric and may-->dysphagia, hoarseness


-Gottron's sign: rash over IP, MCP or elbows


-heliotrope rash on eyelids


-shawl or V sign on chest/back w/ UV exposure


-mechanics hands (associated w/ ILD)


-calcinosis (juvenile form)

complications of dermatomyositis

-associated w/ increased risk for malignancies


-interstitial lung disease associated with anti-synthetase Ab's (Jo-1)

diagnosis of dermatomyositis

-elevated CK and aldolase


-abnormal EMG: increased membrane irritability w/ fibrillations, discharges, low amplitude


-Ab's: ANA, anti-tRNA synthetase, anti-signal recognition peptide, anti-M2


-biopsy: perifascicular inflammation (CD4+) often w/ perivascular involvement

polymyositis

-progressive proximal muscle weakness


-symmetric and may-->dysphagia, hoarseness


-mechanics hands (associated w/ ILD)


-elevated CK and aldolase


-abnormal EMG: increased membrane irritability w/ fibrillations, discharges, low amplitude


-Ab's: ANA, anti-tRNA synthetase, anti-signal recognition peptide,


-biopsy: CD8+ infiltrate into the muscle fibers

Inclusion body myositis

-occurs mostly in older men


-progressive weakness/atrophy often seen in hand flexors and quads


-low elevation of muscle enzymes


-abnormal EMG


-treatment resistant


-rimmed vacuoles w/ mild inflammation seen on biopsy

mimics of inflammatory muscle disease

-Drug induced myopathy: steroids (normal enzymes&EMG), colchicine, hydroxychloroquine, AZT, cocaine, statins and others


-Viral


-Hypothyroidism

properties of amyloid

-non-branching fibrils ~7.5 nm wide


-beta pleated sheet conformation


-stains w/ congo red-->apple green birefringence


-insoluble

components of amyloid

-proteoglycans


-P component


-protein fibrils: either...


AL= Ig light chains (mostly lambda)


AA= serum amyloid associated protein made in liver w/ chronic inflammation


AF/TTR=transthyretin, carrier protein for thyroxine

organs affect by amyloidosis

Kidney: proteinuria-->nephrotic syndrome


spleen: enlargement either 'sago' (white pulp) or lardaceous (diffuse)


liver:enlargement, deposits in space of dis


heart: restrictive cardiomyopathy


bone marrow: may have high plasma cells


tongue swelling

diagnosis of amyloid

-abdominal fat pad aspirate


-gingival/rectal biopsy


-visceral organ biopsy


-serum/urine electrophoresis


-bone marrow biopsy

clinical presentation of AL amyloidosis

-caused by plasma cell dyscrasia or multiple myeloma


-fatigue, weight loss


-multiple organ involvement: kidney, heart, GI, tongue, liver, spleen+ others


treatment of AL amyloidosis


-melphalan or other chemotherapeutics


-autologous stem cell transplant