1. Maggie Davis Hovda, MD 3/22/2010
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
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

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
Intermittent – subsequent flares less severe
Chronic – destructive arthritis

8. 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