Table 2

Frequency of disease worsening

Disease worseningYesNoMissing
Any*228 (32.3)478 (67.7)
Worsening FVC103 (14.6)514 (72.8)89 (12.6)
Death within 12 (±3) months92 (13.0)614 (87)
New echocardiography-suspected PH37 (5.2)582 (82.4)87 (12.3)
New renal crisis7 (1.0)613 (86.8)86 (12.2)
Worsening LVEF5 (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.