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

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

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

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

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 7 sets of criteria proposed, all of which are similar, the above have the highest sensitivity -need 5 different criteria – 2 major, 2 minor Rash = nonpruritic maculopapula or macular rash that is salmon and over the trunk or extremities during febrile illness Joint involvement = knees, wrists, ankles, elbows, PIPs, shoulders; arthritis can become destructive with fusion of wrist joints char of ASD but in a minority of pts LFTs – transaminases abnormal Exclusion – any evidence of infection or malignancy

Laboratory Evaluation Ferritin > 3,000

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 Intermittent – subsequent flares less severe Chronic – destructive arthritis

Treatment NSAIDS – mild disease Steroids – most patients Biologics – TNFa inhibitors, anakinra, rituximab DMARDS – cyclosporine, MTX -with high fevers, debilitating joints, internal organ involvement start with steroids