Adult-Onset Still’s Disease & Relapsing Polychondritis

Adult-onset Still’s disease (J Rheumatol 1992;19:424; Autoimmun Rev 2014;13:708)

  • Rare autoinflammatory synd; ♂ = ♀ w/ typical onset 16–35 y; sx evolve over wks to mos
  • Dx if 5 criteria are present & ≥2 major; exclude infxn, malig, other rheumatic, drug rxn
    • Major: fever ≥39°C for ≥1 wk (usually daily or twice daily high-spiking fever); arthralgias/ arthritis ≥2 wk; Still’s rash (qv); ↑ WBC w/ 80% PMN
    • Minor: sore throat; LAN; HSM; ↑ AST/ALT/LDH; negative ANA & RF
  • Still’s rash (>85%): nonpruritic macular or maculopapular salmon-colored rash; usually trunk or es; may be precipitated by trauma (Koebner phenomenon), warm water