Frequency of disease worsening
| Disease worsening | Yes | No | Missing |
| Any* | 228 (32.3) | 478 (67.7) | — |
| Worsening FVC | 103 (14.6) | 514 (72.8) | 89 (12.6) |
| Death within 12 (±3) months | 92 (13.0) | 614 (87) | |
| New echocardiography-suspected PH | 37 (5.2) | 582 (82.4) | 87 (12.3) |
| New renal crisis | 7 (1.0) | 613 (86.8) | 86 (12.2) |
| Worsening LVEF | 5 (0.7) | 614 (87.0) | 87 (12.3) |
Data are n (%).
*Patients were considered to have disease worsening if death occurred within 12±3 months after baseline or if worsening was present for any of the other components.
FVC, forced vital capacity;LVEF, left ventricular ejection fraction;PH, pulmonary hypertension.