Table 1.
Clinical and demographic features of patients with SJIA-LD*
| All patients (n = 41) | Definite LD (n = 13) | Probable LD (n = 24) | P value (definite vs probable) | |
|---|---|---|---|---|
|
| ||||
| Sex, ratio | 17 M:24 F | 6 M:7 F | 8 M:16 F | 0.49 |
| Age at SJIA diagnosis (IQR), y | 1.5 (0.93–3.38) | 1.5 (1–5) | 1.3 (0.9–3) | 0.47 |
| Age at LD diagnosis (IQR), y | 3 (2–6) | 4 (2–7) | 3 (2–6.3) | 0.64 |
| Disease duration at LD diagnosis (IQR), y | 1.2 (0.7–3) | 1 (0.75–3) | 2 (0.77–3) | 0.60 |
| Clubbing present, % (n/total n) | 78 (32/41) | 85 (11/13) | 79 (19/24) | 1.0 |
| Trisomy 21, % (n/total n) | 15 (6/41) | 23 (3/13) | 8 (2/24) | 0.32 |
| Oxygen requirement ever, % (n/total n) | 37 (15/41) | 46 (6/13) | 38 (9/24) | 0.73 |
| Abnormal overnight oximetry, % (n/total n) | 65 (11/17) | 78 (7/9) | 50 (4/8) | 0.33 |
| MAS, % (n) | ||||
| Never | 15 (6) | 8 (1) | 9 (2) | 1.0 |
| Subclinical | 8 (3) | 15 (2) | 4 (1) | 0.28 |
| One episode | 32 (13) | 38 (5) | 29 (7) | 0.72 |
| >One episode | 46 (19) | 38 (5) | 58 (14) | 0.31 |
| Biologic exposure, % (n) | ||||
| IL-1 | 100 (41) | 100 (13) | 100 (24) | 1.0 |
| IL-6 | 54 (22) | 54 (7) | 50 (12) | 1.0 |
| Adverse event to biologic, % (n/) | 41 (17) | 38 (5) | 38 (9) | 1.0 |
| HLADR5/HLA-DR1*15, % (n/total n) | 84 (21/25) | 100 (11/11) | 75 (9/12) | 0.21 |
| Median follow-up (IQR), y | 2.9 (0.2–15.5) | 3.4 (0.9–6.4) | 2.1 (0.2–15.5) | 0.56 |
| Survival, % (n/total n) | 93 (38/41) | 92 (12/13) | 92 (22/24) | 1.0 |
*
P values are Fisher’s exact test for categorical data, t-test for continuous data. IQR, interquartile range; IL, interleukin; MAS, macrophage activation syndrome; SJIA-LD, systemic juvenile idiopathic arthritis–associated lung disease.