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ODR ICM
Juvenile Idiopathic Arthritis
33
Medical
Graduate
12/15/2010

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Term
systemic onset JA
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)
Term
What are the clinical symptoms of systemic JA?
Definition
arthritis: knees, elbows, MCPs, ankles axillary lymphadenopathy hepatosplenomeg evanescent rash quotidian (1x/day) fever pattern
Term
What condition exhibits a quotidian (1x/day) fever pattern?
Definition
systemic onset JA
Term
What do you seen in lab eval of a patient w/systemic onset JA?
Definition
leukocytosis
anemia
thrombocytosis
^ESR & CRP
^ferritin (correlates w/disease activity)
Term
In what condition do you see increased levels of ferritin which correlates to disease activity?
Definition
systemic onset JA
Term
What are the complications of systemic onset JA?
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
Term
How is systemic onset JA treated?
Definition
IV metylprednisone, cyclosporin A, anti IL1 & IL6
high fatality rate :-(
Term
oligoarticular JIA
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)
Term
In what condition do you see severe erosions & joint space narrowing after 10 years?
Definition
systemic onset JA
Term
What are the clinical symptoms of oligoarticular JA?
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
Term
What JIA primarily effects the lower extremities?
Definition
oligoarticular
Term
In what condition do you see compensatory positions?
Definition
oligoarticular JIA
Term
How is pain elicited in oligarticular JIA?
Definition
extreme ROM
pressure or tenderness, maximal and joint line or inflamed synovium
Term
How is oligoarticular JIA diagnosed?
Definition
chronic arthritis in less than 4 jts
ANA, RF
leg length discrepancy
micrognathia
brachydactyly
Term
In what condition do you see a leg length discrepancy?
Definition
poly/oligoarticular JIA
Term
In what condition do you see micrognathia (small jaw) and brachydactyly (short fingers/toes)?
Definition
poly/Oligoarticular JIA
Term
What are the complications of poly/oligoarticular JIA?
Definition
micrognathia
cerv spine involvement
risk of allantoaxial subluxation & SC compression
Baker's (popliteal) cyst
Term
Which of the JIAs has the best outcome?
Definition
oligoarticular
Term
What are the age groups affected by systemic onset vs oligoarticular vs polyarticular JIA?
Definition
systemic: 4-6yo
oligoarticular: 1-3yo
polyarticular: adolescent onset
Term
Which JIA has the greatest female:male ratio?
Definition
polyarticular (3.5:1)
Term
Which gender is most affected by JIAs?
Definition
females
Term
Polyarticular JIA
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
Term
What are the clinical symptoms of polyarticular JIA?
Definition
adolescent onset
symmetric joint involvement
rheumatoid nodules
rheumatoid vasculitis
Term
In what JIA do you see rheumatoid nodules?
Definition
polyarticular JIA
Term
JIA-associated uveitis
Definition
ANA+ girls are at increased risk
bilateral
frequently asymptomatic
typically diagnosed withing 7yrs of JIA diagnosis
should screen every 3-4 months
Term
What are complications of JIA associated uveitis?
Definition
glaucoma, cataracts, band keratopathy, posterior synechial, vision loss
Term
What are the criteria for enthesis-related disease?
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
Term
List the features of acute anterior uveitis.
Definition
unilateral
painful
redness
tearing
photophobia
Term
Describe the arthritis in enthesitis-related arthritis.
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
Term
In what condition do you see tarsitis?
Definition
enthesitis-related arthritis
Term
What is enthesitis:
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
Term
What is seen in psoriatic arthritis?
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
Term
In what condition do you see dactylitis?
Definition
psoriatic arthritis (sausage fingers)
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