Patient and disease characteristics in 52 patients with JMCTD with and without ILD at examination after 16.2 years disease duration. Values are n (%) or median (range) unless otherwise specified.
| Characteristics | No ILD, n = 38 | ILD, n = 14 | p |
|---|---|---|---|
| At examination | |||
| Female sex | 34 (90) | 10 (71) | NS |
| Age, yrs, mean (SD) | 27.1 (10.3) | 30.4 (10.2) | NS |
| Disease duration, from symptom onset | 15.1 (10.2) | 19.4 (10.2) | NS |
| BMI, kg/m2, mean (SD) | 23.3 (3.0) | 23.7 (2.9) | NS |
| Current smokers | 3 (8) | 4 (29) | NS |
| Never smokers | 26 (68) | 9 (64) | NS |
| SLE-like disease | 22 (58) | 5 (36) | NS |
| SSc-like disease | 22 (58) | 14 (100) | 0.025 |
| PM-like disease | 2 (5) | 0 | NS |
| PFT | |||
| Impaired PFT^ | 27 (73) | 9 (64) | NS |
| DLCO < 80% | 26 (70) | 9 (64) | NS |
| DLCO < 70% | 13 (34) | 6 (43) | NS |
| FVC < 80% | 5 (14) | 6 (43) | 0.023 |
| TLC < 80% | 1 (3) | 5 (36) | 0.002 |
| FEV1/FVC < 0.70 | 1 (3) | 0 | NS |
| 6MWT, m, mean (SD) | 626.5 (77.1) | 626.9 (79.8) | NS |
| Disease activity at examination | |||
| Active disease | 26 (68) | 9 (64) | NS |
| No. active joints | 0 (0–11) | 0 (0–2) | NS |
| Anti-RNP, titer × 103, U/l | 83.5 (0–240) | 234.0 (17–240) | NS |
| ESR, mm/h | 9.0 (2–54) | 8.0 (2–30) | NS |
| CRP, mg/l | 0.7 (0–15) | 0.7 (0–9.3) | NS |
| Anti-Ro52-positive | 1 (3) | 0 | NS |
| PGA, 10 cm VAS | 1.5 (0–58.0) | 2.9 (0.2–5.7) | NS |
| Rodnan skin score | 0 (0–4) | 0 (0–11) | NS |
↵^ FVC, FEV1, and/or DLCO < 80% of predicted, and/or FEV1/FVC < 0.7. JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease; BMI: body mass index; SLE: systemic lupus erythematosus; SSc: systemic sclerosis; PM: polymyositis; PFT: pulmonary function test; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; TLC: total lung capacity; 6MWT: 6-min walk test; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; PGA: physician’s global assessment; VAS: visual analog scale; NS: not significant.