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328 Cards in this Set
- Front
- Back
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define OA
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deterioration of articular cartilage
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causes of 2ndry oseoarthritis?
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another rheumatic dz, trauma, neurologic disorder, metabolic dz (hypothyroidism, acromegaly)
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2 fxns of articular cartilage
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smooth gliding and load support
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type of collagen in articular cartilage? Role
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type II, tensile trength by binding proteoglycan
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role of proteoglycan
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component of collagen that is hydrophilic and produces resistance to compression
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early and late changes of collage in OA?
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early- synshtesis increased w/ abnormal fibers; late- synthesis and content decreased
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early and late changes of proteoglycan in OA?
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early-synthesis inc w/ smaller subunits/less aggregation; late- synthesis/content decreased
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water content in OA?
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decreased
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dz: hip bone shows an irregular surface, multiplied chondrocytes, and less collagen
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OA
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t/f inflammation causes OA?
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false- inflammation late and minimal
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Dz:joint pain w/ use or motion/loss of motion; 10 minutes stiffness after resting, constant
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OA
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Dz: bony enlargement of joint, minimal tenderness, limited range of motion, crepitus, asymmetry
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OA
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t/f generalized weakness in OA?
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false- atrophy of adjacent musce
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t/f effusions in OA?
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true- low cell count, non-inflammatory
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common joints of OA
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hand- first CMC, PIP, DIP (no wrist, MCP), cervical/lumbar spine, hip, knee, first MTP
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uncommonly involved in OA
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shoulder, thoracic spine, elbow, wrist, ankle, subtalar
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t/f OA is symettric?
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FALSE
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t/f loss of joint space w/ OA?
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TRUE
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dz: xray shows sclerosis of bone on each side of CMC
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OA
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which dz is caused by pointed toes/high heelps?
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osteoarthritis of MTP
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hallux valgus?
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distal part of toe points lateral. Can be single manifestation of OA.
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valgus vs. verus?
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valgus= lateral; verus= medial
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what disease can cause OA of the hip and needs to be caught early on?
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epiphyseal dysplasia
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Dz: bony spurs of lumbar spine, scoliosis?
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OA
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laboratory findings in OA?
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none- not an inflammatory process
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steps of management of OA?
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1. life style 2. acetaminophine 3. intermitent low dose nsaid 4. full dose scheduled nsaid
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indications for PT in Oa?
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loss of joint motion but preserved structure; muscle weakness w/ joint instability; severe sx
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t/f people w/ OA of hip/knee should exercise?
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true, decreases disability
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if someone has osteoarhritis and uses a cane, which hand is cane in?
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opposite
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intra-articular therapy for OA?
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corticosteroid (methylprednisolone), hyaluranon
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topical therapy for OA?
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capsaicin P, NSAIDS
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when to do orthopedic surgery in OA?
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failed other tx, major lifestyle restrictions, motivated patients
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orthopedic surgeries in OA?
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arthroplasty-partial or total; arthrodesis, fusion; osteotomy/realigment
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late joint structure changes in Oa?
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synovial hypertrophy, joint effusion, remodeling
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Pathogenesis of OA
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physical stress=chondrocyte damage=release of degradative enzyme= lost strength=microfracture=bone stiffer= loss of compressibility
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age of symptomatic OA?
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usually startsin 50s, everyone has asymptomatic changes
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what sex gets OA more? Where?
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Women- also earlier and at a higher frequency- knee, hip, hand
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mechanism of cox release
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phospholipase cleaves membrane bound phospholipids=AA=Cox=prostaglandins
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which Cox is most proinflammatory?
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cox2
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synthesis of cox 1? Cox2?
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cox-1= constituitive, cox-2= inducible
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lipocortin?
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upregulated by corticosteroids; blocks phospholipase causing less coversion to AA and less cox
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cortisol is short of long acting? How much Na retention?
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short; lots
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mechanism of corticosteroids?
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steroid-receptor interaction blocks NF-kB action
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what do corticosteroid supress? What do they enhance?
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suppress-proinflammatory cytokines/enzymes, neutrophil migration, lymphocyte fxn; enhance- lipocortin (less AA)
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metabolic adverse efects of corticosteroieds
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elevated- glc, lipids, Na retention; redistributed body fat, impair childrens growth; 2ndry adrenal insufficency
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Dz: pt w/ cushingoid appearance/ buffalo hump
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chronic stroid use
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msk adverse effects of corticosteroids?
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osteoperosis (inc clast, dec absorption/)/kyphosis; steroid myopathy; impaired wound healing; avascular necrosis
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non-msk and non-metabolic advese effects of corticosteroids?
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infection, cataracts, peptic ulcers w/ nsaid, acne, altered mood/behavior
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t/f steroid dose and infection rate are does dependent?
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TRUE
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absorption and distribution of nsaids?
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complete absorption, distributes to all tissues and his highly protien bound
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clearance of nsaids?
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hepatic metabolism, renal or fecal clearance
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when to reduce nsaid dose
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renal/liver dz, CHF, low albumin, extreme age
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nsaid renal affects
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reversible renal dysfunction;reversible hyperkalemia, interferes w/ efficacy of diuretics, rare intersitital nephritis
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GI complications w/ NSAIDs? Risk factors?
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ulcer; OTC, anticoagulants, corticosteroids, over 60 smoking
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t/f H pylori infection risk factor for NSAID GI complications?
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FALSE
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nsaid ulcers are different from h. pyori ulcers how>
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gastric, asymptomatic (bloody urine), no inflammation
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nsaid cns effects?
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HA, aseptic meningitis, confusion/lethargy, tinnitus
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nsaid hemorrhagic effects and risk factors?
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impaired platelet aggregation, anemia, rare thrombocytopenia/neutropenia; don't take Ibuprofen before asprin may block protective effect of asprin
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t/f nsaids cause an allergy?
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true: w/ asthma history, nasal polyps, or family hx
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vioxx vs. naproxen?
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vioxx= 1/2 gi bleeds but more MI
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celecoxib vs. nsaid?
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cox 2 inhibitors, fewer GI bleed unless asprin is also used
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dz:loss of lumbar odosis, exaggerated cervial lordosis, bent knees and hips
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spondylitis
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who gets spondulitis?
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males 3:1, onset age 20-40 max
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t/f people w/ ankylosing spondylitis should exercise?
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true: improves w/ exercise
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morning stiffness in akylosing spondylitis?
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prominent
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onset of ankylosing spondylitis?
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insidious, has to las > 3 months
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t/f tenderness in ankylosing spondylitis?
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TRUE
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schober test
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measure abilit to flex lumar spine
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occiput to wall test
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neck flexed in AS
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non-msk features of AS?
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iritis, conjuctivitis, aortic insuficcency
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dz: eye hurst moving from light to dark?
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AS-iritis
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early changes in As reflect ensopathy or synovitis? Late?
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early-enthesopathy, late- synovitis
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t/f erosions in AS? Sclerosis? Fusion?
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TRUE
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early and late radiogrphic spines of AS?
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shiny corner (erosions) sign and bamboo spine (bony bridges)
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t/f anti-TNF chanes dz progression in AS? why?
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false- wNT and BMP pathway are involved in syndesmophyte formations, not just TNF
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main tx for spondyloarthropathies?
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NSAIDs, particullary indocin
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define reactive arthritis?
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seronegative asymmetric arthropathy predominantly in lowe extremities + inflammatory eye dz, mucocutaneous dz, dysentry, urethritis/cervicitis
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organisms in reactive arhritis?
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salmonella, shigella, yersina, campylobacter, chlymydia, kebsiella
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dz:4 joints w/ back pain and red eyes, pain bearing weight on plantar fascia
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reactive arthritis
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how many joints involved in reactive arthritis?
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4 or less, assymetric
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dz: sausage digit, diffusely swollen
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reactive or psoriatic arthritis
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dz: conjuctivitis and monarthritis?
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reactive arthritis
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dz: blistering/hyperkeratotic/peeling feet
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keratodermia blenorrhagica of reactive arthritis
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dz: circular raised, weepy, hyperkeratotic penis
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circinate balantis of reactive arthritis
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dz: linqual erosions?
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reactive arthritis
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dz: raising of nails from bed
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onycholysis of reactive arthritis
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tx for eactive arhtritis?
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indocin, doxycycline if due to chlamydia
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reactive arthritis can be presenting symptom of what dz? How is tx different?
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HIV; may respond poorly to nsaids; methotrexate contraindicated
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dz: scaly hyperkeratosis on elbow and bely button?
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psoriasis
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who gets psoriatic arhtritis
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10% of psoriatic pts, age 20-40, both sexes
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most common pattern of psoriatic arhritis?
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assymetric oligoarhtitis (i.e. sausage digit)
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dz: symmetric polyarhtitis only
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symmetric polyarhtritis of psoriatic arhritis
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4 least common patterns of psoriatic arthritis?
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symmetric poly arthritis, DIP arthritis w/ nail dz, arthritis mutilans, spondylitis
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dz: arthritis w/ nail and dIP involvement?
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DIP arthritis of psoriatic arhtritis
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dz: nail pits?
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psoriatic arhtritis
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dz: pencil in cup deformity on xray?
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arthritis mutilans of psoriatic arthritis
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t/f extent of skin disease predicts extent/severity of arthritis?
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FALSE
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psoriasis usually occurs before or after the arthritis?
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usually before-80%
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issues in psoriatic arthritis tx?
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steroids make skin worse, antimalarias cause severe rash, avoid arthrocentesis through plaque
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tx for psoriatic arhtritis? Role
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methotrexate/TNF blockers- inbrove both
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enteropathic arthritis is associated w/
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dz that cause bacterial overgrowth-Chron's, UC, bypass, diverticular dz
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who gets enteric arthritis?
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20% of IBD patients, both sexes, age 25-45
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dz: aerythema nodosum?
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enteric arhritis
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describe enteric arthritis
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oligoarticular, LE
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t/f peripheral arthritis goes away w/ colectomy in enteric arthritis? Spondylitis?
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truve; false
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tx of enteric arthritis?
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sulfasalazine; TNF blcokers- joints and bowels
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what medicine to avoid in enteric arhtritis?
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misprosotol, nsaids used w/ caution
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2 times to think spondyloarthropathy?
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1. assymetric synovitis + enselopathy, cervicitis, diarrhea, esarcoilitis, alternate buttox pain, IBD/psoriasis OR inflammtory spinal pain > 3 months w/ lots of morning back stiffness
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most common disc degeneration/herniation?
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L4/5> L5/S1
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prevalence of low back pain?
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affects 85% of populatind uring lifetime, 26% occurrence per year
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90% of back pain caused by
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degenerative changes, traumatic fracture, soft tissue injury
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low back pain red flags?
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FAINT= fracture, infection (IV drug use, wt loss, fever, immunosupression), tumor, nerve injury, age (>50)
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how to manage low back pain w/ no red flags?
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time, anti-inflammatories/muscle relaxants, Jarvick-LAID back study
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when to image in low back pain?
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red flag, no response to treatment, acute worsening/non-responsive chronic pain
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utility of xrays for low back pain?
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low yield for acute lbp, but oblique xrays show scotty dog sifn for pars defect (fractured neck)
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conservative treatments for low back pain?5
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back exercise, nsaid, muscle relaxant,narcotic, oral steroids
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low back paih dz where surgery is exclusive treatment?
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cauda equina syndrome
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dz/tx: primarily low back pain, muscle spasms
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mechanical low back pain; conservative, injections; rarely steroids
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dz/tx: unilateral leg pain, numbness, or weakness
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radiculopathy; conservative and injections unless weakness. Weakness/debilitating pain consider surgery
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l4/l5 central disc hernation affects what nerve root?
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L5
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dz/tx: severe bilateral leg pain and distal weakness. Urinary retention or incointenence. True motor weakness & increased post void residue
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cauda equina syndrome(large disc herniation of lumbosacral nerve roots); tx- urgen surgery
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dz/tx: legs tired w/ activity, grocerry cart sign, sx better lying down or walking uphil?
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lumbar stenosis/neurogenic claudication. Conservative management, maybe injection or surgery
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severe degeneration of facets or pars defects leads to what?
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spondylolithesis- slipping forward fo verterbal body
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define spondylosis?
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arthritic degenerative changes of spine
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dfine spndylolysis?
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defect in pars interarticularis w/ out movement
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define spondylolithesis?
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displacement/slipped vertebrae, slid forward
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grades of spondylolithesis?
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i=25%, 2=up to 50, 3= up to 75, 4=100, more than 100= spondyloptosis
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define the pars interarticularis
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part between the facets- can not form (congenital pars defect) or be injured w/ trauma (pars fracture)
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which low back pain dz causes scotty dog appearance on oblique xray?
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spondylolysis
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what is the most dangerous joint dz?
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nongonoccocal bacterial arthritis
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who gets septic arhtritis?
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RA pts w/ prostehtic joints, 70/80 yo, equal in both sexes
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most common pathophys of septic arthritis? Other cuases?
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1-hematogenous from distal site; 2 from a focus of osteomyelitis, lymphogenic from skin infection, iatrogenic, pentratic trauma
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who gets contiguous septic arthritis?
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children; <1 b/c blood vessels communicate
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how long until irreversible changes occur in septic arthritis?
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damage w/ in hours, irreversible in days (7)
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most important organism in septic arhtritis?
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staph aureus
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organism of septic arthritis in prostetic joints?
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staph aureus, CNS, skin anerobes/gram negative bacilli (particulary shoulder- back acne)
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organism of septic arthritis in alcoholism/cirrhosis?
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gram-negative bacilli
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location/organism of septic arthritis in drug abusers?
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sternoclavicular/sacroilliac; pseudomonas
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organism of septic arthritis in DM?
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gram positive and negative
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highest risk factors for septic arthritis? Others?
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skin infection + prostetic hip, DM, over 60 or less than 1, prostethics/surgery, RA, IVDA
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clinical features of septic arthritis?
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joint pain, swelling +/- fever
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describe nongonococcal septic arthritis?
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abrupt onset, usu monoarticular: knee, hip, anlke most common)
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Dz: sudden, hot, swollen painful, tender knee, hip, or shoulder
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nongonococcal septic arthritis
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risk factors for gonococcal septic arthritis?
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STD risk factors, young females, pH changes-menstruation, pregnanccy
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differences in portal of entry for gonococcal septic arthritis?
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pharyngeal and rectal more likely to be asymptomatic and culture negative
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skin rash (papule), migrating joint pain in knees and wrists, and inflammation around tendon,fever?
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arthritis dermatitis syndrome of gonococcal septic arthritis
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2 forms of gonococcal septic arthritis? Which is more likely to have positive blood cultures?
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arthritis-dermatitis bacteremic syndrome-cultures; localized septic arthrittis
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dz:mono or poly joint pain; fever
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septic arthritis of gonococcal arthritis
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best lab test for septic arthritis?
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synovial fluid directly into blood culture bottle: >50 k WBC w/ over 90% neutrophils, low glc, high LDHL
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dz: cloudy synovial fluid, >50k WBC w/ more than 90% neutrophils
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which form of septic arthritis is more likely to be graim stain or culture positive?
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50--80% in non gonoccocal; less than 20 of gonococcal
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describe n. gonorrhea?
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fastidious organism that grows on chocolate argar (non contaminated) or tahyer martin medium (contaminated)
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source of infection of nongonococcal septic arthritis?
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urine, sputum, wound, blood
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source of infection of gonococcal septic arthritis?
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genital, rectal, pharyngeal
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when is PCR good for diagnosing septic arthritis?
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fastidious organism, but not gonococcal; not staph/strep
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use of imaging in septic arthritis?
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not good for diagnoses-CT guides aspiration, MRI evaluated complication, Radionuclide scans differentiate bone and surrounding soft tissue inflammations
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general tx of septirc arthritis?
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joint drainiage, abx, early joint mobilization
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when to do surgical drainage of spetic arhtritis?
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hip, poorly accesible joints, suspected osft tissue extension; inadequate clinical response
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septic arthritis antibiotic if gram stain negative? Length?
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cover staph and strep IV for a few weeks; if suspect gonococoal 3rd generation cephalosporin- Ceftriaxone, Cefotaxime oral a few days
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define times of presthetic joint infection
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early-3 months, delayed-24 months, late >24 months
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early prostetic joint infection organisms?
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virulent- staph aureus, gram negative bacilli
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delayed prostetic joint infection organisms?
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less virulent- CNS, acne, anerobic gram positive in shoulder
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dz: feels like prostehtic loosening, mild swelling, mild temperature increase
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delayed septic prostetic arthritis
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organisms of late prosthetic joint infec?
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acute- virulent staph aureus, GAS/GBS; E. coli; chronic- indolent CNS, acne
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which septic arthritis has best prognosis?
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gonococcal
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dz: kid w/ trouble walking, worse in morning, not painful, rash, flexed knees
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JIA/JRA
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criteria for JRA?
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less than 16, over 6 weeks, swelling/effurion + 2: limited ROM, pain w/ ROM, tenderness, increased heat
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who gets JRA?
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peak age onset= 1-4, 3x girls until 9, then equal
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dz: knees, ankle, elbow artritis, kid, blond hair, girl, uveitis
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pauci-articular onset juveline arthritis
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predict course of pauci articular onset arthritis?
|
if ANA += great outcome, uveitis more servere; RF+=poor outcome
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tx of pauci-articular arthritis?
|
nsaids, ocas. Injections, if refractory/severe uveitis- high dose methotrexate
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complications of Pauci-JIA
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different elg lengths, fixed flexion contractures, uveitis (20%)
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dz: mispannen pupul, WBC/inflammation in anterior chamber in eye w/ synechiae? Complications?
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veitis in JRA; cataracts, glaucoma, blindness
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tx of uveitis?
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steroid, mydriatics
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dz: 5 or more joings, symmetric arthritis including fingers and toes, not painful, kid?
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polyarticular onset juvenile arthritis
|
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when is morbidiity highest in polyarticular onset juvenile arthritis?
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late age onset, RF +, feamles
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dz: TMJ
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polyarticular onset juvenile arthritis
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uveitis is common in which JRA
|
pauciarticular
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complications of polyarticular JRA?
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early osteoarthritis, joint destruction/subluxation, madalum/vshaped deformity of fused wrist, one short toe, flexion contractures of fingers, bunyans, cervical spine limitations, small jaw (TMJ)
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tx: polyarticular JRA?
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anti-tnf, nsaids, injections, steroids, methotrexate
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dz: high fever, visceral disease, salmon-conored macular rash
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systemic onsent JRA/still's dz
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which joint is hard to tx and almost always involve in systemic juvenile arthritis?
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wrist
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tx: still's dz/ systemic Jra
|
anti-IL-6, nsaid, injections, less anti TNF
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lab test in systemic JRA?
|
elevated sed rate, nocrmocytic, hypochromic anemia, leuocytosis, trhombocytosis; no RF! Only for prognosis
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radiographic changes of systmic JRA?
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soft tissue swelling, widening of joint space followed by narrowing, erosions, and subluxation
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who gets slipped capital femoral epiphysis?
|
during growth spurt, more amles, obese, blacks/pacif islanders
|
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radiographic changes of slipped capital femoral epiphysis?
|
Klein's line is off
|
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define legg-calve-perthres?
|
idiopathic osteonecrosis of femoral head due to occlusion of a single artery
|
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who gets legg- calve-perthes?
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males, whites, 3-12
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dz: isolated hip effusion after infection in 1 leg?
|
toxic synovitis
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tx for toxic synovitis?
|
aspirate hip
|
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who gets benign hypermobility syndrome?
|
very flexible people, an overuse syndrome
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t/f connective tissue is abnormal in benign hypermobility syndrome?
|
FALSE
|
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who gets bening nocturnal pains of childhood?
|
school adged kids: 4-12
|
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dz: nightime crampy deep pain in lower extremeties, worse w/ exercise, better w/ massage?
|
benign nocturnal pains of childhood
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cause of acute rheumatic fever?
|
Group A beta hemolytic strep
|
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criteria for acute rheumatic fever?
|
2 major or 1 of each: major-carditis, polyarthritis, erythema marginatum, subq nodules, chorea, minor- arthralgia, fever, elevated sed, prolonged PR
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time most common for ARF?
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spring
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dz: migratory painful arthritis of knees, ankles, wrist elbows; better w/ asprin
|
acute rheumatic fever
|
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dz: macular serpinginous rash on trunk?
|
erythema marginatum of ARF
|
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carditis in ARF involves what valve?
|
mitral valve
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dz: subcutaneous freely moveable nodules on elbow?
|
chronic rheumatic heart disease
|
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dz: 30 yo woman w/ no blood pressure in left arm
|
takayasu's arteritis
|
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large cessel vaculitis?
|
giant cell arteritis, takayausu arteritis
|
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medium vessel vasculitis?
|
PAN, Kawasaki's dz
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define takayasu's arteritis?
|
granulomatous inflammation of aorta and major branches
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who gets takayuasu's arteritis?
|
asian women, <50
|
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define giant cell arteritis?
|
granulomatous arteritis of aorta and major brances especially carotids/temporal
|
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who gets giant cell arteritis?
|
over 50, men
|
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giant cell arteritis is associated w/ which dz?
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polymalgia rheumatica
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dz: tender touching somewhere on face, shade over eye
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giant cell temporal arteritis
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what disease can lead to blindess?
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giant cell temporal arteritis and pauci-immune JIRA
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dz: scalp necrosis
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gaint cell temporal arteritis
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dz: retinal ischemia )cotton wool exudates on retinal exam)
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giant cell arteritis: retinal ischemia
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dz: weakness of shoulder area arms in eldely?tx?
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Pplymaylagia rheumatica; high dose corticosteroid if includes temporal arteritis
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how to dx takayasu's?
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angiography
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how to diagnose giant cell arteritis?
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temporal artery biopsy
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polyarteritis nadosa is associated w/ which dz?
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hepatitis B
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define polyarteritis nodosa?
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necrotizing inflammation of medium or small sized artery w/ out glomeruloneprhitis
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dz: skin nodules, wrist drop, orchitis, microaneurysms in kidneys?
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polyarteritis nadosa
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dzs: subq nodules
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acute rheumatic fever, polyarteritis nadosa
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dz: slightly raised macular rash, kid, chapped lips, strawbery tongue, skin desquamasation?
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kawasaki dz
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biggest complication of kawasaki dz?
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coronary artery aneurysm--> MI
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define kawaski's dz?
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mucocutaneous lymph node syndrome in kids, complicated by coronary arteritis
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extraarticular dz in rheumatoid arthritis?
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vasculitis, pulmonary nodules, effusion, and interstital disease, eye ulcers or sjoren's syndrome
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define HSP?
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vasculitis w/ IgA dominant Immne deposits; involves skin, gut, glomeruli
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define essential cryogloulinemic vasculits; associated w/ what dz?
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vasculitis w/ cryo deposints involves skin and glomeruli, associated w/ hep C
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define wegne'rs granuloma
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graulomatous inflammation of rspiratory tract w/ necrotizing vasculitis and glomeruloneprhtitis
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define churg-staurss syndrome?
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eosinophilc, granulomatous inflammation of respiratory tract and necrotizing vasculitis associated w/ asthma
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define microscopic polyangitis?
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necrotizing vasculitis w/ no immune deposis that may affect large arteries
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which small cell vasculitis don't affect the lung?
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HSP and cryoglobulinemia
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Which small cell vaculitis don't affect the ent area/gave sinusiits?
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HSP and cryoglobulinemia
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dzs: palpable purura on legs?
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small cell vasculitis
|
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dz: reticular patern of erythema?
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cryoglobulinemia
|
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dz; digital gangrene/raynaud's
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small cell vasculitis, epscryoglobulinemia
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dz: saddle nose
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wegner's granuloma
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dz: sinus opacification
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wegner's granuloma
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dz: cANCA? Direct against what?
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wegners, agianst proteinase 3
|
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dz: pANCA? Directed against what?
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Churg-strauss syndrome/microscopic polyangiits; myeloperoxidase
|
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dz: astma eosinophilia?
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churg-strauss
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dzs: immune complexes
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HSP and cryoglobulinemia
|
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dz: small cell vasculitis w/ large artery involvement
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microscopic polyangitis
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lab findings of vasculitis?
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high ESR, severe anemia, thrombocytosis, por renal fxn
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|
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how to diagnose vasculitis?
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image of chest and sinus, SCG to rule out vegetations, biopsy area
|
|
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in which vaculitis is angiograph helpful?
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takayasu's ; PAN (Sacular aneurysm on mesenteric angiogram)
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|
|
tx of vasculitis?
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corticosterooids*, cytotoxic drugs: methotrexate, aza, cyclosporine; IV Ig for kawasaki's
|
|
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complication of cyclophosphamide in tx of vasculation?
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cystitis, cancer, bone marros suppresion
|
|
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name 3 infectious etiologies of vasculitis
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1. eBV/herpes virus association 2. syphilitic aortitis looks like takuysau's aortitis 3. hepatitis C causes autoantibodies to make cryo
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tx of cryoglobulinemia?
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reduce viral load, immunosupression if necessary
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who gets lupus?
|
black and asian women age in reproductive years
|
|
|
list 11 classifications of lupus. Need 4
|
1. immuno disorder 2. ANA 3. hematologic disorder 4. arthritis 5. neurological disorder 6.photosensitivity 7. malar rash 8. discoid rash 9. ora ulcers 10. serositis 11. renal disorder
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how to distinguish malar rash from rosaceua?
|
rosacea involves nasolabial folds, rosacea has papules that look like acne
|
|
|
dx lupus rashes?
|
deposition of Ig or C at lupus band test
|
|
|
t/f oral ulcers in lupus are painful?
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FALSE
|
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distinguish lupus arthritis from rheumatoid arthritis
|
no erosions
|
|
|
define serositis?
|
pleuritis- pain or rub Or pericarditis
|
|
|
indications fo renal disorder in lupus?
|
proteinuria, cellular casts
|
|
|
different treatment indication w/ histo forms of lupus?
|
tx type IV diffuse proliferative aggresively; type 6 sclerosing is not treated aggressively b/c it is so bad
|
|
|
neurologic disorders found in lupus?
|
seizures or psychosis, stroke, mental status changes, myelopathy
|
|
|
hematologic disorders in lupus?
|
hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
|
|
|
what type of anemia is present in lupus?
|
autoimmune hemolytic anemia
|
|
|
what causes keukopenia nd lymphopenia in sle?
|
peripheral overdestruction of (not inadequate production!)
|
|
|
how does thrombocytopenia present in SLE?
|
immune thrombocytopenic purpura (ITP)
|
|
|
name 2 things than may be presenting manifestation of lupus?
|
cytopenias (anemia, ITP) or arhralgias
|
|
|
immunologic disorder of lupus?
|
anti-DNA or anti-Sm or antiphospholipid antibodies (Ig, lupus anticoagulant-non-correctingprolonged PTT, syphilis)
|
|
|
use of ANA for lupus daignosis?
|
very sensitive, not specific
|
|
|
most informative ANA pattern in lupus? What leads away from lupus diagnosis
|
homogenous anti dsDNA; anti-histone seen in drug induced lupus
|
|
|
role of procainamide, quinidie, hydralazine in lupus?
|
drug induced lupus- + ANA, more benign, anti-histone ANA
|
|
|
what causes neonatal lupus?
|
anti-ro and anti-la autoantibodies in mother
|
|
|
dz: infant w/ complete heart block
|
neonatal lupus
|
|
|
dzs: sojuren's syndrome?
|
arhtritis, SLE
|
|
|
dzs: raynauds?
|
lupus, cryoglobulinemia, scleroderma
|
|
|
how to distinguis SLE rash from dermatomyositis?
|
SLE spares joints (gottron's is on knuckles)
|
|
|
if suspsicion for lupus is low, how do you order lab evaluations?
|
panel 1: less specific, CBC, ana, etc.
|
|
|
if suspsicion for lupus is high, how do you order lab evaluations?
|
Panel 2: dDNA, ro/la, {TT
|
|
|
use of kidney biopsy in SLE?
|
establish involvement, predict prognosis- not used ind x
|
|
|
complement in lupus?
|
complement is involved via deffective immune complex handling or immune response (coombs, band test) but complement deficicents predispose-not protect
|
|
|
why are genome wide association studies not good for lupus?
|
linked dz alleles are common in general population; oddsratio is low
|
|
|
name tx for lupus
|
ansaid, antimalarias, dapsone, corticosteroids, aza, methotrexate, mycophenolate, cyclophosphamide
|
|
|
which lupus tx are good for cutenaneous lupus?
|
antimalarias and dapsone
|
|
|
2 types of systemic scleroderma
|
diffuse and limited
|
|
|
who gets scleroderma?
|
morewomen, age 30-50
|
|
|
dz: puffy shinny hands that can't close
|
scleroderma
|
|
|
what is acrosteolysis? In what dz?
|
shortening finger tips from boney remodeling; scleroderma
|
|
|
dz: digital calcinodis?
|
scleroderma
|
|
|
jackhammers vibration can cause what?
|
raynauds
|
|
|
how idiopathic primary raynaud's different than systemic sclerosis?
|
it doesn't have thickened endothelium so it isn't as severe and doesn't lead to ischemia
|
|
|
dz: digital pitting scars (not on nails)
|
scleroderma
|
|
|
dz: dilated vessels of nailfold capillaries?
|
scleroderma
|
|
|
dz: esophageal dysmotility on barium swallow?
|
scleroderma
|
|
|
t/f lung changes in scleroderma?
|
true- interstitial lung dz
|
|
|
worst complication fo scelroderma? Signs?
|
renal crisis- hypertension, microangioapathic hemolytic anemia, risking Cr/K, pulmonary edema, death
|
|
|
dz: ANA centromere pattern
|
limited scleroderma
|
|
|
what does crest stand for?
|
Calcinosis Reynauds Esophageal motility Scerodactyl Telangiectasia= limited scleorderma
|
|
|
in which form of scleroderma is there telangiectasis?
|
crest
|
|
|
in which form of scleroderma is there calcinosis?
|
crest
|
|
|
in which form of scleroderma is there tendon rubs
|
diffuse
|
|
|
in which form of scleroderma is there pulmonary htn?
|
crest
|
|
|
in which form of scleroderma is there renal crises?
|
diffuse
|
|
|
in which form of scleroderma is there ACA?
|
crest
|
|
|
what drug is used to prevent reflux in scleroderma?
|
all must be on a PPI
|
|
|
gi symptoms in scleroderma?
|
hypomotility leading to bacterial overgrowth/diarrhea
|
|
|
describe how microvascular occlusion leads to scleroderma?
|
endothelial cells activate IL-1= increased adheasion-->Altered balance of prostacyclin an thromboxane--> platelet activation--> tgf-b--> stiulates endothelin--> vasoconstrction and fibroblast synthesis
|
|
|
describe pathogenesis of fibrosis in scleroderma?
|
activate fibroblast--> secrete collagen and proliferate w/ high synthetic genotype; increased TIMP-1, a protocollagenase inhibitor= decreased remodling of collagen= accumulation of extracellular matrix= organ dysfxn
|
|
|
immune cell activation in pathogenesis of scleroderma
|
humoral- hyperglobulinemia, autoAb; cellular- T cells activated and transforming, macrophages-activated and secreting cytokines; mast cells- increase in number stimulating fibroblast and damaging endothelial cells
|
|
|
inflammatory problems in scleroderma?
|
alveolitis, myositis, thendonitis
|
|
|
define polymyositis?
|
acquired idiffuse inflammatory disorder of sketaltal muscle
|
|
|
define dermatomyositits
|
polymyositis accompanied by characteristic rash
|
|
|
who gets pm/dermatomyositis?
|
5-15 more males; 45-65 more females
|
|
|
muscle weakness in dermatomyositis?
|
proximal (climbing stairs, getting out of chair), symmetric, insidious onset, dysphagia, dysphnia
|
|
|
dz: heliotrope rash
|
dermatomyositis
|
|
|
dz: shawl pattern rash?
|
dermatomyositis
|
|
|
dz: rash on extensor surfaces of knucles?
|
gottron's papules of dermatomyositis
|
|
|
dz: prominent nail fold capillaries?
|
dermatomyositis
|
|
|
dz: calcinosis poking through skin?
|
dermatomyositis
|
|
|
emg changes on dermatomyositis?
|
never reach maximum activity
|
|
|
lab changes in PM/dermatomyositis?
|
emg changes, muscle biopsy-de/regernation, lymphocyte infiltration,increased enzymes (CK,aldolasE), positive ANA
|
|
|
when to see enzyme changes in pm/dm
|
before clinical remisions and exacerbations
|
|
|
t/f active myositis is treatable
|
TRUE
|
|
|
autoantibodies in myositis and their clinical significance?
|
Jo-1 anti synthetase shows up w/ mechanics hands, interstitial lung disease, fever, and arthritis
|
|
|
dz: hyperkeratotic, peeling, split hands
|
mechanic's hands of Jo-1 dermatomyositis
|
|
|
drugs that can cuase weakness?
|
corticosteroids, statins-CK will be normal, non-inflammatory
|
|
|
infections that can cause weakness?
|
viral, esp HIV
|
|
|
principles of therapy for pm/Dermatomyositis?
|
1. high dose prednisone 2. taber every month by 25% if CK normal 3. continue a year 4. watch out for steroid myopathy 5. consider cytotozic i.e. aza for resistant dz
|
|
|
sign of steroid myopathy
|
pt gets recurring weakness but CK stays the same
|
|
|
inflammatory mechanism in polymositis
|
cell medaited Ag specifc cytotoxicity: cd8 cells in endomysium, MCH class I expression on myocytes
|
|
|
inflammatory mechanisms in dermatomyositis?
|
humorally-mediated muscle fiber damage: b cell infiltrated, MAC complex deposited in vessel walls
|
|
|
4 abnormalitie s of myocyte fxn in dermatomyocites
|
1. destruction of fibers 2. ATP-metabolism disrupting contraction/membranes 3. tNF inhibits contraction, promotes catabolism 4. capillary dropout, hypoperfusion= Muscle Weakness
|
