Table 1.
Baseline characteristics of patients with sJIA and treatment overview.
| Clinical information | Patients with sJIA (n = 76) |
|---|---|
| Demographics | |
| Age at diagnosis, years (range) | 4.5 (0.6–14.1) |
| Sex, % female (n = male, female) | 53% (36, 40) |
| Clinical manifestations | |
| Fever, n (%) | 76 (100%) |
| Rash, n (%) | 69 (91%) |
| Lymphadenopathy, n (%) | 37 (49%) |
| Hepatomegaly, n (%) | 11 (14%) |
| Splenomegaly, n (%) | 17 (22%) |
| Serositis, n (%) | 11 (14%) |
| Arthritis, n (%) | 61 (80%) |
| Active joint count, n = 70 (range) | 4 (0–26) |
| MASa, n (%) | 27 (36%) (confirmed, n = 20; suspected, n = 7) |
| Laboratory investigations | |
| ESR (mm/h), n = 48 (range) | 120 (10–170) |
| CRP (mg/L), n = 54 (range) | 95 (5–400) |
| Ferritin >500 μg/L (n = 59) | 37 (63%) |
| Haemoglobin <9 g/dl (n = 59) | 14 (24%) |
| Platelets >600 × 109/L (n = 61) | 18 (30%) |
| sJIA classification criteria and systemic manifestation score | |
| Met the classification criteria for sJIA | 59 (78%) |
| Systemic manifestation scoreb (n = 66) | 4 (0–9) |
| Treatment received within 3 months of diagnosis | |
| Oral/IV glucocorticoids (%) | 70 (92%) |
| Methotrexate (%) | 36 (47%) |
| IL-1 blockade (anakinra)c, n (%) | 21 (28%) |
| IL-6 blockade (tocilizumab)c, n (%) | 6 (8%) |
| Other immune modulationd, n (%) | 22 (29%) |
| Treatment at last review | |
| Time since diagnosis, years (range) | 4.7 (0.2–16) |
| Oral/IV glucocorticoids (%) | 6 (8%) |
| Methotrexate as sole DMARD (%) | 8 (11%) |
| Current anakinra or tocilizumabc, n (%) | 38 (50%; n = 20 anakinra; n = 18 tocilizumab) |
| Other treatmente, n (%) | 14 (18%) |
| Biologic everf, n (%) | 64 (84%) |
Median values presented along with range. Absolute frequencies presented along with percentages.
MAS at presentation. This has been subdivided to report number of children that presented with confirmed MAS, i.e., fulfilled the 2016 MAS classification criteria (25); and suspected MAS, i.e., where a diagnosis of MAS was documented in the electronic record, but without full documentary evidence satisfying the classification criteria.
SMS quantifies burden of systemic symptoms on a 0–10 scale. Includes five clinical elements (fever, evanescent rash, generalised lymphadenopathy, hepatomegaly and/or splenomegaly, and serositis) and two laboratory elements (anaemia, defined as Hb < 9 g/dl, and either platelet count >600 × 109/L or ferritin >500 ng/ml). Each item, if present scores 1, apart from fever which is further characterised as follows: temperature 37°C–38°C (scores 1), 38°C–39°C (scores 2), 39°C–40°C (scores 3), and >40°C (scores 4) (23).
Irrespective of concomitant sDMARD or immunosuppressant.
Treated with an alternative agent(s): cyclosporine n = 12, intravenous immunoglobulin n = 6, emapalumab n = 2, etoposide n = 1, and etanercept n = 1.
Treated with an alternative agent(s): cyclosporine n = 3, adalimumab n = 3, HSCT n = 4, canakinumab n = 2, etanercept n = 1, and colchicine n = 1.
Received treatment with a biologic DMARD at some point throughout their disease course: IL-1 blockade, n = 35; IL-6 blockade, n = 26; IL-1 and IL-6 (sequentially), n = 3; anti-TNF, n = 8). IV, intravenous; IL, interleukin.