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 #Adult #onset #Stills #disease #AOSD #diagnosis #rheumatology #management
