Adult-Onset Still's Disease (AOSD)
Rare inflammatory disorder of unknown etiology, difficult to diagnose because of heterogenous presentation.
Must rule out other likely conditions (autoimmune, infectious, malignant) to diagnose.
Pathogenesis: Unclear, but innate immune response implicated. Elevated IL-18, IL-6, TNF-a, ? Genetic susceptibility, infectious triggers
Presentation: High spiking fever, evanescent salmon-colored maculopapular rash, arthritis, neutrophilic leukocytosis. Life-threatening complications in 15-20%
Monocyclic: single episode (30%)
Systemic AOSD: high fever, CRP, AST+ALT, ferritin. Innate immunity (30%)
Chronic articular AOSD: F>M, low ferritin, arthritis and chronic synovitis (40%)
Treatment: NSAIDs, steroids (acutely), DMARDS (steroid-sparing), IVIG, cyclophosphamide, biologics
- BWH Medicine Chiefs @BrighamChiefs
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