Term
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Definition
preceded by 2 weeks of fever and at least 1 of the following? evanescent, non-fixed erythematous rash, lymphadenopathy, hepatosplenomegaly, serositis mean age: 4-6 yo affects any joints F:M (1.5:1) |
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Term
What are the clinical symptoms of systemic JA? |
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Definition
arthritis: knees, elbows, MCPs, ankles axillary lymphadenopathy hepatosplenomeg evanescent rash quotidian (1x/day) fever pattern |
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Term
What condition exhibits a quotidian (1x/day) fever pattern? |
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Definition
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Term
What do you seen in lab eval of a patient w/systemic onset JA? |
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Definition
leukocytosis anemia thrombocytosis ^ESR & CRP ^ferritin (correlates w/disease activity) |
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Term
In what condition do you see increased levels of ferritin which correlates to disease activity? |
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Definition
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Term
What are the complications of systemic onset JA? |
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Definition
macrophage activation system: macrophages expand uncontrolably when NK cells don't work rapid development of hepatic failure w/enceph purpura, bruising, mucosal bleeding assoc w/renal failure persistent fever leukopenia, anemia, thrombocytopenia, decreased ESR cytokin storm |
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Term
How is systemic onset JA treated? |
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Definition
IV metylprednisone, cyclosporin A, anti IL1 & IL6 high fatality rate :-( |
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Term
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Definition
F:M (3:1) peak age 1-3 arthritis w/uveitis seldom familial no more than 4 jts in first 6 months (persistent: never > 4, extended: >4 over time) |
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Term
In what condition do you see severe erosions & joint space narrowing after 10 years? |
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Definition
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Term
What are the clinical symptoms of oligoarticular JA? |
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Definition
not systemically ill predominanly LEs: one or both knees, ankles; can affect small jts of fingers/toes but less common pain at extreme ROM aching/stretching pain elicited by pressure or tenderness, maximum at joint line or inflamed synovium compensatory positions |
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Term
What JIA primarily effects the lower extremities? |
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Definition
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Term
In what condition do you see compensatory positions? |
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Definition
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Term
How is pain elicited in oligarticular JIA? |
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Definition
extreme ROM pressure or tenderness, maximal and joint line or inflamed synovium |
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Term
How is oligoarticular JIA diagnosed? |
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Definition
chronic arthritis in less than 4 jts ANA, RF leg length discrepancy micrognathia brachydactyly |
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Term
In what condition do you see a leg length discrepancy? |
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Definition
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Term
In what condition do you see micrognathia (small jaw) and brachydactyly (short fingers/toes)? |
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Definition
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Term
What are the complications of poly/oligoarticular JIA? |
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Definition
micrognathia cerv spine involvement risk of allantoaxial subluxation & SC compression Baker's (popliteal) cyst |
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Term
Which of the JIAs has the best outcome? |
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Definition
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Term
What are the age groups affected by systemic onset vs oligoarticular vs polyarticular JIA? |
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Definition
systemic: 4-6yo oligoarticular: 1-3yo polyarticular: adolescent onset |
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Term
Which JIA has the greatest female:male ratio? |
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Definition
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Term
Which gender is most affected by JIAs? |
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Definition
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Term
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Definition
F:M (3.5:1) RF (+) on at least 2 ocasions separated by 3 months arthritis in at least 5 joints in the first 6 months adolescent onset |
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Term
What are the clinical symptoms of polyarticular JIA? |
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Definition
adolescent onset symmetric joint involvement rheumatoid nodules rheumatoid vasculitis |
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Term
In what JIA do you see rheumatoid nodules? |
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Definition
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Term
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Definition
ANA+ girls are at increased risk bilateral frequently asymptomatic typically diagnosed withing 7yrs of JIA diagnosis should screen every 3-4 months |
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Term
What are complications of JIA associated uveitis? |
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Definition
glaucoma, cataracts, band keratopathy, posterior synechial, vision loss |
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Term
What are the criteria for enthesis-related disease? |
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Definition
arthritis & enthesitis or arthritis or enthesitis with at least 2: SI jt tenderness/inflamm spinal pain HLA b27 family hx of b27-related disease acute anterior uveitis onset of arthritis in boy >8yo exclusions: psoriasis or sytemic features |
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Term
List the features of acute anterior uveitis. |
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Definition
unilateral painful redness tearing photophobia |
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Term
Describe the arthritis in enthesitis-related arthritis. |
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Definition
lower extremities isolated hip disease is common tarsitis: pain and decreased motion of midfood toes commonly involved but not fingers SI joint involvement spinal involvement |
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Term
In what condition do you see tarsitis? |
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Definition
enthesitis-related arthritis |
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Term
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Definition
inflammation of tendons/ligaments at their attachment sites to bones common sites: 2,6, & 10 o'clock around patella tibial tuberosity achilles tendon attachment at calcaneous plantar fascia attachment at calcaneous heads of metatarsals base of 5th metatarsal |
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Term
What is seen in psoriatic arthritis? |
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Definition
hidden psoriatic lesions: nail pitting, navel & gluteal crease + family hx of psoriasis may look like pauci/oligoarticular but labs are worse (high inflamm markers) dactylitis (sausage fingers) prognosis is generally worse |
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Term
In what condition do you see dactylitis? |
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Definition
psoriatic arthritis (sausage fingers) |
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