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29 Cards in this Set
- Front
- Back
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ACR criteria for juvenile rheumatoid arthritis(JRA)?
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-arthritis in one or more jt
*arthritis defined as swelling or effusion and at least two of limited ROM, pain w/ ROM, tenderness of jt on palpation, increased heat -age at onset <16 yr -duration of disease >6 wks ***exclusion of other forms of juvenile arthritis (sarcoidosis, SLE, ARF, post infectious, PVNS, JDM, acroosteolysis syndrome) |
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3 types of JRA?
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systemic
polyarticular pauciarticular |
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girls or boys.. with more prevelance of JRA?
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girls
MF = 1:3 |
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JRA usually starts with what type?
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pauci articular
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typical presentation of pauci articular juvenile arthritis?
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stiff in the morning, crawl for hr, then walk with limp
no pain, fever, rash or systemic Sx swollen knee can't straighten leg |
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If ANA+ in pauci articular? how is the outcome?
any associated Sx w/ ANA+ ? |
outcome excellent
ANA+ related to uveitis |
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how's the outcome for RF+ pts in pauci articular?
what if seronegative? (RF and ANA) |
poor if RF+
difficult to predict if seronegative |
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Tx of Pauci articular?
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NSAIDS - ibuprofen, naproxen
Injection - triamconolone hexacetonide physical therapy Systemic Tx if refractory or severe uveitis: MTX, anti TNF |
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complications of pauci?
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localized growth disturbances
fixed flexion contractures uveitis |
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what's uvea?
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iris, ciliary body , choroid
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what's uveitis?
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chronic, non granulomatous inflammation
often aSx pain, redness, HA, photophobia, and changes in vision synechiae, cataract, glaucoma, blindness may precede the onset of arthritis by years |
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Rx of uveitis?
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steroids, mydriatics
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top 2 causes of uveitis in children?
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1. idiopathic
2. JRA |
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describe poly articular onset of juvenile arthritis
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40% of JRA
**5 or more joints involved symmetric arthritis of large and small joints fingers, toes, wrists, neck, spine elevated ESR, platelet, mild anemia morning stiffness ** no pain TMJ arthritis common and refractory to Rx uveitis less common |
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which group of polyarticular onset JRA has highest disability?
common age for polyarticular? |
late age onset, RF +
two peaks: 1-3 yrs, teens |
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which polyarticular onset pts have good outcome?
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ANA +
toddler girls.. |
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how's the outcome of seronegative pts in polyarticular JRA?
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hard to predict
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complications of polyJRA
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same as pauciarticular
localized growth disturbances fixed flexion contractures uveitis in addition, joint destruction/subluxation early osteoarthritis |
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Tx of polyarticular onset?
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same as pauciarticular
add steroid to systemic therapy |
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what's still's disease?
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**high spiking fever
evanescent, salmon colored macular non pruritic rash visceral disease: hepatosplenomegaly, lymphadenopathy, serositis, pericarditis, vasculitis, myocarditis, pneumonitis elevated acute phase reactant, marked leukocytosis, thrombocytosis, anemia systemic involvement may precede development of arthritis |
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describe systemic juvenile arthritis (still's disease)
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arthritis is polyarticular
wrists are sep difficult to reat, and almost always involved. appear ill w/ fever, well w/o fever half remit, half recurrent 2-4% mortality |
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what pt group has poor outcome in still's?
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poly arthritis and erosions
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what's the hallmark of still's?
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quotidian fever
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other features of still's?
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growth failure
micrognathia |
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Tx of still's?
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same as pauci, polyarticular JRA
in addition CSA/mychophenolate/AZA anti-IL1(anakinra) anti IL-6 **bone marrow transplant |
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pathogenesis of JRA?
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autoimmune disease - Tcell, autoantibody, synovitis, immune complex, complement activation
genetic - HLA association, twin concordance environmental triggers - numerous reported, none proven |
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lab tests of JRA?
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acute phase reactants elevated
normocytic, hypochromic anemia leukocytosis and thrombocytosis in systemic onset disease ANA, RF for prognosis not Dx RF specific but not sensitive |
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synovial fluid analysis of JRA?
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inflammatory - WBC 1000-100,000
avg 11,000 predominantly neutrophils low viscosity immune complexes |
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DDx of JRA?
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slipped capital femoral epiphysis (SCFE)
Legg-calve-perthes (LCPD) acute rheumatic fever toxic synovitis bening hypermobility syndrome growing pains |
