Baseline demographic and clinical features of the study patients considered as a whole and divided by response to IL-1 inhibition.
| All Patients, n = 62 | Complete Responders, n = 24 | Nonresponders, n = 38 | p | |
|---|---|---|---|---|
| Sex: male | 32 (52) | 13 (54) | 19 (50) | 0.749 |
| Age at disease onset, yrs, median (IQR) | 4.9 (2.7–9.8) | 6.2 (2.2–9.5) | 4.3 (2.8–10.9) | 0.745 |
| Age, yrs, median (IQR) | 9.7 (4.1–13.1) | 8.8 (3.3–12.8) | 10.8 (4.3–13.0) | 0.326 |
| Disease duration, yrs, median (IQR) | 1.4 (0.4–5.5) | 0.9 (0.2–2.5) | 2.1 (0.6–6.3) | 0.082 |
| Concomitant medications | 57 (92) | 24 (100) | 33 (87) | 0.147 |
| Corticosteroids dose, mg/kg/day, median (IQR) | 0.5 (0.2–1.0) | 0.7 (0.3–1.0) | 0.5 (0.2–1.0) | 0.655 |
| MTX | 18 (29) | 6 (25) | 12 (32) | 0.578 |
| Patients with active arthritis | 46 (74) | 16 (67) | 30 (79) | 0.282 |
| Active joint count, median (IQR) | 3 (0–10) | 2 (0–4) | 4 (1–12) | 0.056 |
| Systemic manifestations | 53 (85) | 22 (92) | 31 (82) | 0.462 |
| Fever | 48 (77) | 19 (79) | 29 (76) | 0.794 |
| Rash | 25 (40) | 11 (46) | 14 (37) | 0.482 |
| Hepatomegaly | 13 (21) | 6 (25) | 7 (18) | 0.535 |
| Splenomegaly | 5 (8) | 3 (13) | 2 (5) | 0.366 |
| Generalized lymphadenopathy | 5 (8) | 3 (13) | 2 (5) | 0.366 |
| Serositis | 4 (6) | 4 (17) | 0 (0) | 0.019 |
| Hemoglobin < 9 g/dl | 9 (14) | 5 (21) | 4 (10) | 0.29 |
| Platelet count > 600 × 109/l or ferritin > 500 ng/ml | 37 (60) | 17 (71) | 20 (53) | 0.15 |
| Systemic manifestation score, median (IQR)* | 3 (2–4) | 4 (2.5–5) | 3 (2–3) | 0.018 |
| PGA, median (IQR) | 8 (7.5–9) | 8.2 (7.5–9) | 8 (7.5–9) | 0.401 |
| WBC count, × 109/l, median (IQR) | 16.2 (12.1–24.5) | 20.8 (14.1–27.3) | 15.7 (11.7–20.3) | 0.103 |
| Neutrophil count, × 109/l, median (IQR) | 10.7 (7.9–18.4) | 16.6 (8.1–23.7) | 9.6 (7.9–16.2) | 0.125 |
| Hemoglobin, g/dl, median (IQR) | 10.6 (9.6–12.0) | 10.7 (9.1–11.8) | 10.6 (9.7–12.3) | 0.589 |
| Platelet count, × 109/l, median (IQR) | 536 (382–644) | 535 (412–645) | 536 (370–644) | 0.796 |
| CRP, mg/dl, median (IQR) | 9.7 (5.3–14.5) | 9.6 (4.5–13.8) | 9.7 (5.6–14.5) | 0.729 |
| ESR, mm/h, median (IQR) | 61.5 (51–77) | 60 (50–77) | 62 (52–78) | 0.767 |
| Ferritin, ng/ml, median (IQR) | 364 (118–1436) | 615 (145–2925) | 274 (113–533) | 0.038 |
| Previous therapies | ||||
| Glucocorticoids | 61 (98) | 24 (100) | 37 (97) | 1.000 |
| MTX | 38 (61) | 12 (50) | 26 (68) | 0.147 |
| Anti-TNF agents | 23 (37) | 5 (21) | 18 (47) | 0.035 |
Data are represented as n (%), unless otherwise indicated.
↵* Systemic manifestation score (0–10 points). IQR: interquartile range; PGA: physician’s global assessment of disease activity; MTX: methotrexate; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; WBC: white blood cell; anti-TNF: tumor necrosis factor antagonists; IL: interleukin.