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General Reference

Jama 2005;294:1671

Pathophys and Cause

Pathophys:(J Rheum 1990;21[suppl 21]:1)

Epidemiology

Incidence = 1.4/10 000 children/yr; prevalence = 1/1000 children in US

Signs and Symptoms

Sx/Si:

Polyarticular type (45%): onset at later age, occasionally rheumatoid factor positive; arthritis in image5 joints; no systemic sx or si

Pauciarticular type (30%); arthritis in <4 joints; often ANA positive; iridocyclitis leads to blindness often even when arthritis is inactive; eye disease often asx (Clin Exp Rheum 1990;8:499)

Systemic type (25%): intermittent fever <39.4°C (103°F), no arthritis; Still’s disease variant: diurnal fevers, salmon-colored reticular rash, high ESR, pleuropericarditis, but rare or late arthritis

Course

20% still impaired at 10 yr

Complications

Growth impairment (Clin Orthop 1990;259:46)

r/o, if age appropriate (Rheum Rev 1991;1:13): juvenile ankylosing spondylitis with positive HLA B27 (Bull Rheum Dis 1987;37[1]:1); child abuse; neoplasms; infections (viral, endocarditis, Lyme disease); granulomatous disorders (Crohn’s, sarcoid); connective tissue diseases like PAN, SS, giant cell arteritis, rheumatic fever, and SLE

Lab and Xray

Lab:

No good diagnostic test

Hem:Anemia and leukocytosis

Serol:ANA often positive in pauciarticular type. HLA studies 90% positive for B27, cf Reiter’s and ankylosing spondylitis (juvenile type only)

Treatment

Rx:

(Clin Orthop 1990;259:60)

Physical therapy (Rheum Dis Clin N Am 1991;17:1001)

NSAIDs like ibuprofen; intra-articular steroids; these are enough in 50-60%, esp the pauciarticular type

Methotrexate up to 1 mg/kg/wk, usually 0.5 mg/kg/wk up to 25 mg, better than leflunomide (Nejm 2005;352:1655)

Tumor necrosis factor antagonists (Disease Modifying Antirheumatic Drugs (DMARD)) like adalimumab (Humira) IgG1 recombinant monoclonal antibody to TNF; 20-40 mg IM q2weeks based on weight. OK to continue aminosalicylates, corticosteroids, and/or immunomodulatory agents.