Characteristics | Complete Responders, n = 28 | Nonresponders, n = 17 | p |
---|---|---|---|
Female | 16 (57.1) | 9 (52.9) | 0.78 |
Age at symptom onset, yrs | 4 (0.75–15.7) | 5 (1–4.6) | 0.46 |
Age at diagnosis, yrs | 4.3 (0.9–15.9) | 5.3 (2–13) | 0.46 |
Age at initiation of anakinra, yrs | 6.5 (1.6–16.1) | 8 (2.8–13.2) | 0.49 |
Disease duration at initiation of anakinra, mos | 6 (1–142) | 24 (2–84) | 0.17 |
Dose of anakinra, mg/kg daily | 2 (2–4) | 2 (2–5) | 0.46 |
PGA, 0–10 scale, 10 worst | 5 (2–7) | 5 (4–8) | 0.84 |
Fever | 28 (100) | 17 (100) | — |
Rash | 22 (78.6) | 11 (64.7) | 0.32 |
Hepatomegaly | 9 (32.1) | 6 (35.3) | 0.82 |
Splenomegaly | 11 (39.3) | 5 (29.4) | 0.54 |
Generalized lymphadenopathy | 11 (39.3) | 4 (23.5) | 0.27 |
Serositis | 7 (25) | 4 (23.5) | 1 |
Hemoglobin, g/dl | 10 (7.6–15.1) | 10.3 (6.5–12.6) | 0.72 |
WBC count, × 103/mm3 | 12.75 (2.4–52.9) | 11.3 (4–21) | 0.26 |
Neutrophil count, × 103/mm3 | 8.45 (0.6–43.6) | 8.5 (1.9–18.6) | 0.78 |
Platelet count, × 103/mm3 | 480 (219–883) | 504 (202–820) | 0.59 |
ESR, mm/h, normal range 0–20 | 46 (10–120) | 49 (28–120) | 0.81 |
CRP, mg/dl, normal range 0–0.8 | 9.41 (1–34) | 7.18 (1.6–26) | 0.26 |
Fibrinogen, mg/dl, normal range 144–430 | 409.5 (206–983) | 406.5 (238–1200) | 0.74 |
Ferritin, ng/ml, normal range 11–307 | 620.5 (312–8849) | 162 (50–692) | 0.056 |
Systemic manifestation score | 5 (2–8) | 3 (2–8) | 0.13 |
Duration of anakinra treatment, mos | 17 (3–60) | 10 (2–50) | 0.26 |
Still using anakinra at 1 yr | 24 (85.7) | 7 (41.2) | 0.002 |
Still using corticosteroids at 1 yr | 1 (3.6) | 14 (82.4) | < 0.001 |
Presence of flare under anakinra treatment | 4 (14.3) | 15 (88.2) | < 0.001 |
Numeric variables are presented as median (min–max) values. Nominal variables are presented as n (%). CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; PGA: physician’s global assessment; WBC: white blood cell.