Table 2.
Clinical Features of EOSD and AOSD.
| EOSD n=25 |
AOSD [Ref. 7, 35-45] |
|||
|---|---|---|---|---|
| Japanese patients, n (%) | 17/25 (68) | 168/940 (18) | ||
| Epidemiology | ||||
| Mean age, years old | 76.6±4.9 | 24-46 | ||
| Female, n (%) | 18/25 (74) | 45-75% | ||
| Clinical features | ||||
| Fever, n (%) | 25/25 (100) | 85-100% | ||
| Arthritis, n (%) | 19/25 (76) | 72-100% | ||
| Typical rash, n (%) | 17/25 (68) | 58-94% | ||
| Leukocytes ≥10,000/μL, n (%) | 21/25 (84) | 72-98% | ||
| Neutrophils ≥80%, n (%) | 16/21 (76) | 55-89% | ||
| Sore throat, n (%) | 16/25 (64) | 27-92% | ||
| Lymphadenopathy, n (%) | 9/25 (36) | 28-74% | ||
| Splenomegaly, n (%) | 7/25 (28) | 29-79% | ||
| Elevated liver enzymes, n (%) | 21/25 (84) | 21-76% | ||
| Negative for rheumatoid factor, n (%) | 21/22 (95) | 93-100% | ||
| Negative for antinuclear antibodies, n (%) | 21/21 (100) | 89-100% | ||
| Complications | ||||
| MAS, n (%) | 5/25 (20) | 12-21% | ||
| DIC, n (%) | 5/25 (20) | 1.8-6.3% | ||
| Infection, n (%) | PCP 2/25 (8) CMV 4/25 (16) |
NA | ||
| ARDS, n (%) | 1/25 (4) | NA | ||
| Interstitial pneumonia, n (%) | 1/25 (4) | NA | ||
| Multi-organ dysfunction, n (%) | 1/25 (4) | NA | ||
| Cerebellar hemorrhage, n (%) | 1/25 (4) | NA | ||
| Subacute hepatitis, n (%) | 1/25 (4) | NA | ||
| Malignancy, n (%) | 1/25 (4) | NA | ||
| Inflammatory myopathy, n (%) | 1/25 (4) | NA |
EOSD: elderly-onset Still’s disease, AOSD: adult-onset Still’s disease, MAS: macrophage activation syndrome, DIC: disseminated intravascular coagulation, ARDS: acute respiratory distress syndrome, PCP: pneumocystis pneumonia, CMV: cytomegalovirus, NA: not available