Table 2.
Occurrence of novel severe organ involvement and/or progression during follow-up
| Novel severe organ involvement and/or progressiona | Follow-up period |
||
|---|---|---|---|
| 0–12 months | 12–24 months | Entire follow-up 0–24 months | |
| (n = 320) | (n = 301) | (n = 334) | |
| Novel peripheral vascular involvement, n (%) | 65 (20.3) | 50 (16.6) | 111 (33.2) |
| Novel pulmonary involvement | |||
| Structural | |||
| New ILD, n (%) | 10 (3.1) | 9 (3.0) | 19 (5.7) |
| Progression of known ILD, n (%) | 36 (11.3) | 42 (14.0) | 67 (20.1) |
| Vascular | |||
| New PH, n (%) | 24 (7.5) | 21 (7.0) | 45 (13.5) |
| New PAH, n (%) | 1 (0.3) | 4 (1.3) | 5 (1.5) |
| Skin progression, n (%) | 75 (23.4) | 72 (23.9) | 140 (41.9) |
| Death, n (%) | 1 (0.3) | 4 (1.3) | 5 (1.5) |
| Novel heart involvement, n (%) | 9 (2.8) | 9 (3.0) | 18 (5.4) |
| Novel gastrointestinal tract involvement, n (%) | 4 (1.3) | 1 (0.3) | 5 (1.5) |
| Novel SRC, n (%) | 2 (0.6) | 3 (1.0) | 5 (1.5) |
| Novel musculoskeletal involvement, n (%) | 33 (10.3) | 29 (9.6) | 59 (17.7) |
a
Novel severe organ involvement and/or progression is defined as new involvement of any organ system at a future visit (i.e. 12 or 24 months) that did not exist at enrolment or at the prior visit. Of 334 unique SSc patients, 257 (76.9%) had novel severe organ involvement and/or progression. ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; SRC: scleroderma renal crisis.