Adult Onset Still's Disease
Systemic inflammation with urticarial rash.
Rash: 
 • Nonpruritic, evanescent (correlates with time of fever), and salmon- colored!
 • Distribution is often on the chest/trunk
Symptoms:
 • Daily high spiking fever - Fever spikes occur once or twice a day, usually in the late afternoon or early evening
 • Arthritis, arthralgias
 • Lymphadenopathy
 • Leukocytosis
Diagnosis - Yamaguchi criteria is the most widely used for diagnosis - patients must fulfill 5 of the below criteria, 2 of which should be major:
 1. Fever of at least 390C (102.20F) lasting at least one week
 2. Arthralgias or arthritis lasting two weeks or longer
 3. A nonpruritic macular or maculopapular skin rash that is salmon-colored in appearance and usually found over the trunk or extremities during febrile episodes
 4. Leukocytosis (10,000/microL or greater), with at least 80 percent granulocytes
 5. Minor criteria include a sore throat, LAD/splenomegaly, liver dysfunction, and negative RF and ANA
Labs:
 • ↑ESR/CRP
 • Leukocytosis (10,000/microL or greater), with at least 80 percent granulocytes
 • Ferritin >3000 ng/mL Gf consider MAS spectrum)
 • ↑ Plts
 • ↓Hgb
 • High ferritin is characteristic, and the higher the ferritin, the greater the disease activity!
Treatment - Mild: NSAIDS
Treatment - Severe:
 • Prednisone 0.5-1mg/kg/d (may not respond).
 • If uncontrolled: MTX, anti-TNF, anti-lL6R, anti-ILI

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