Characteristics | SoJIA (n = 20) | AoSD (n = 15) |
Gender (M/F) | 8/12 | 4/11 |
Age, years (mean (SD)) | 12.4 (5.2) | 38.1 (12.8) |
Disease duration, years (mean (SD)) | 7.0 (4) | 7.8 (6.4) |
Systemic features (no. of patients) | 12 (60%) | 13 (87%) |
Fever | 9 (45%) | 13 (87%) |
Evanescent rash* | 8 (40%) | 8 (53%) |
Serositis | 3 (15%) | 2 (13%) |
Previous treatments with DMARDs (no. of patients): | ||
MTX† | 19 (95%) | 15 (100%) |
Anti-TNFα | 14 (70%) | 10 (67%) |
Thalidomide | 5 (25%) | 2 (13%) |
IVIG | 0 (0%) | 5 (33%) |
Cyclosporin | 6 (30%) | 0 (0%) |
Other DMARDs‡ | 2 (10%) | 6 (40%) |
Ongoing predniso(lo)ne daily dose | 0.50 (0.32) mg/kg | 26.8 (20.1) mg |
*In three patients with SoJIA, evanescent rash was present in the absence of fever.
†The average and maximum doses of MTX received prior to anakinra treatment were 0.6 mg/kg/week and 1 mg/kg/week, respectively. The average and maximum doses of MTX received prior to anakinra treatment were 20 mg/week and 30 mg/week respectively.
‡Rituximab (n = 1) and cyclophosphamide (n = 1) for SoJIA patients; rituximab (n = 2), cyclophosphamide (n = 1), mycophenolate mofetil (n = 1), azathioprin (n = 1) and sulphasalazin (n = 1) for AoSD pateints.
AoSD, adult onset Still disease; DMARDs: disease-modifying antirheumatic drugs; IVIG, intravenous immunoglobulins; MTX, methotrexate; SoJIA, systemic onset juvenile idiopathic arthritis; TNF, tumour necrosis factor.