Still’s disease is a variant of juvenile idiopathic arthritis that is characterized by seronegative chronic polyarthritis in association with a systemic inflammatory illness. It was initially described in 1897 by George F. Still, a pathologist. The characteristic features of this illness have subsequently been reported in adults, as detailed by Eric Bywaters in 1971.
Systemic illness characterized by quotidian fevers, transient rashes, and an inflammatory polyarthritis.
No specific test is diagnostic but a ferritin level >1000 ng/mL is common.
Macrophage activation syndrome is a severe, life-threatening complication occurring in 5% to 10% of patients.
Nonsteroidal antiinflammatory drugs and corticosteroids control 50% of patients with 33% going into remission.
Methotrexate and biologics are required for 50% of patients including the 33% with a chronic disease course.
Sign and Symptoms of Adult-Onset Still’s Disease:
98% to 100%
83% to 100%
84% to 98%
88% to 94%
50% to 92%
87% to 90%
Weight loss (>10%)
19% to 76%
48% to 74%
45% to 55%
23% to 53%
9% to 48%
29% to 44%
24% to 37%
9% to 31%
Five or more criteria including two or more major criteria yields a 96% sensitivity and 92% specificity to classify a patient as having AOSD.
- Major criteria
- Fever >39 °C for >7 days.
- Arthralgias or arthritis ≥2 weeks.
- Characteristic rash.
- Leukocytosis (≥10,000/μL with ≥80% neutrophils).
- Minor criteria
- Sore throat.
- Hepatomegaly or splenomegaly.Abnormal
- Negative rheumatoid factor and antinuclear antibody.