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Adult Still’s Disease

Adult Still’s Disease

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Adult Still’s Disease

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  1. Adult Still’s Disease Maggie Davis Hovda, MD 3/22/2010

  2. About • Adult equivalent to Juvenile RA • Diagnosis of exclusion • Characterized by: Fevers, arthritis, rash

  3. Etiology • Unknown • Suggestions • Infectious: Viral, Bacterial (mycoplasma, yersinia) • Genetics: certain HLA markers associated with disease, but none substantively so

  4. Epidemiology • No gender preference • Two age peaks • 15 – 25 • 36 – 46

  5. Classification Criteria • Yamaguchi Criteria • 5 total: 2 major + 2 minor • Major • Quotidian Fever > 39 for at least 1 week • Arthralgias/Arthritis at least 2 weeks • Rash • Wbc > 10,000 with PMN predominance • Minor • Pharyngitis • LAD • Abnormal LFTs • Negative ANA, RF • Other: pericarditis, pleural effusions, Reactive HS

  6. Laboratory Evaluation • Ferritin > 3,000

  7. Clinical Course • Monophasic: symptoms < 1 year • Intermittent: more than one flare with total symptom remission in between • Chronic: chronic, active disease • Predictors: develop polyarthritis early in course, shoulder and hip involvement, > two years of glucocorticoid therapy

  8. Treatment • NSAIDS – mild disease • Steroids – most patients • Biologics – TNFa inhibitors, anakinra, rituximab • DMARDS – cyclosporine, MTX