Adult Onset Still’s Disease (AOSD) - Diagnosis and ...

Adult Onset Still’s Disease (AOSD) - Diagnosis and Management Summary

Systemic inflammatory disorder characterized by fevers, arthritis, and rash. Can present as single episode (wks-mos), multiple flares, or be persistently active.

 • Epi: W = M. Bimodal  15-25 yrs old and 36-46 yrs old

 • Sx: fever; arthralgias; evanescent, salmon-colored maculopapular rash that coincides w/ fever, usually on the trunk,

may be precipitated by trauma (Koebner phenomenon); pericarditis; pleural effusions; macrophage activating syndrome

 • Dx: Yamaguchi criteria  requires >5 features, including >2 major criteria (J Rheumatol 1992;19:424):

    o Major: Fever ≥39ºC for ≥1 week, arthralgias/arthritis ≥2 weeks, salmon-colored rash, ↑ WBC (≥10K + ≥80% PMN)

    o Minor: Sore throat, LAD, HSM, ↑AST/ALT, ↑ LDH, negative ANA/RF

    o Other labs (not part of criteria): ↑ ESR/CRP, ferritin >3000 ng/mL (if >10,000, consider MAS spectrum), ↑ plt, ↓ Hgb

 • Rx:

    o Mild: NSAIDs

    o Severe: Prednisone 0.5-1mg/kg/d (may not respond). If uncontrolled: MTX, anti-TNF, anti-IL6R, anti-IL1

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