Demographics and baseline characteristics of the 25 patients. Values are median (IQR) unless otherwise specified.
| Characteristics | Values | ULN/LLN |
|---|---|---|
| Demographic features | ||
| Female, n (%) | 12 (48) | |
| Age at disease onset, yrs | 5.8 (2.9–9.3) | |
| Age at treatment start, yrs | 7.3 (4.8–10.8) | |
| MAS at disease onset, n (%) | 7 (28) | |
| Baseline features | ||
| Fever, n (%) | 23 (92) | |
| Rash, n (%) | 19 (76) | |
| No. active joints | 3 (2–7) | |
| Concomitant glucocorticoids, n (%) | 14 (56) | |
| Concomitant DMARD, n (%)* | 9 (36) | |
| Previous biologics, n (%)** | 6 (24) | |
| CRP, mg/dl | 13.6 (8.6–15.8) | 96% > ULN |
| ESR, mm/h | 82 (65–100) | 100% > ULN |
| Ferritin, ng/ml | 719 (305.5–2875.5) | 71% > ULN |
| WBC count, × 103/µl | 14.7 (8.2–20.3) | 52% > ULN |
| Neutrophil cell count, × 103/µl | 10.5 (6.0–15.5) | 52% > ULN |
| Hemoglobin, g/dl | 10.7 (9.5–11.1) | 80% > LLN |
| Platelet count, × 103/µl | 427 (384–610) | 40% > ULN |
| Time from onset to receiving anakinra, mos | 4.9 (1.6–24.5) | |
| Anakinra dose, mg/kg/day | 2.0 (1.3–2.0) | |
| Anakinra dose ratio administered/ideal† | 1.3 (0.9–1.7) | |
| Outcome | ||
| No. patients with inactive disease at 6 mos from the beginning of the therapy, n (%) | 14 (56) |
↵* Included 6 methotrexate and 3 cyclosporine.
↵** Included 4 etanercept; 1 etanercept and abatacept; 1 etanercept and infliximab.
↵† Ideal as predicted by Figure 4 of Urien, et al14. IQR: interquartile range; MAS: macrophage activation syndrome; DMARD: disease-modifying antirheumatic drugs; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; WBC: white blood cell; ULN: upper limit of normal; LLN: lower limit of normal.