Transcript Adult Still`s Disease

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