from: rheumatology

adult-onset Still’s disease

Adult-onset Still’s disease (J Autoimmun 2018;93:24)

• Rare autoinflammatory syndrome, <4/million per y incidence;
• ♂ = ♀ w/ bimodal typical onset 15-25 or 36-46 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 extremities;
  • may be precipitated by trauma (Koebner phenomenon), warm water
• Plain films: soft tissue swelling (early) → cartilage loss, erosions, carpal ankylosis (late)
• Treatment:
  • NSAIDs;
  • steroids;
  • steroid-sparing (MTX, anakinra, anti-TNF, tocilizumab)
• Variable clinical course: 20% w/ long-term remission; 30% remit-relapse; ~50% chronic (esp. arthritis); ↑ risk of macrophage activation syndrome (life threatening)
• Relapsing polychondritis