Baseline clinical characteristics of the 236 patients with SSc
| Clinical factors | All (n=236) | PAP trajectories | ||||
| Rapid progression (n=9) | Early elevation (n=30) | Middle elevation (n=54) | Late elevation (n=24) | Low stable (n=119) | ||
| Age of onset (years) | 61 (49–69) | 73 (55–74) | 67 (61–71) | 65 (58–70) | 51 (45–58) | 58 (48–67) |
| Female | 204 (86) | 8 (89) | 27 (90) | 42 (78) | 22 (92) | 105 (88) |
| Ever smoking | 65 (28) | 4 (44) | 9 (30) | 16 (30) | 6 (25) | 30 (25) |
| Hypertension | 81 (34) | 5 (56) | 13 (43) | 24 (44) | 8 (33) | 31 (26) |
| Type of autoantibody* | ||||||
| Anti-centromere | 142 (60) | 4 (44) | 13 (43) | 38 (70) | 12 (50) | 75 (63) |
| Anti-topoisomerase I | 47 (20) | 1 (11) | 7 (23) | 12 (22) | 7 (29) | 20 (17) |
| Anti-RNA polymerase III | 13 (5.5) | 2 (22) | 3 (10) | 1 (1.9) | 1 (4.2) | 6 (5.0) |
| Diffuse cutaneous SSc | 58 (25) | 2 (22) | 13 (43) | 14 (26) | 8 (33) | 21 (18) |
| Lung fibrosis | 82 (35) | 6 (67) | 16 (53) | 21 (39) | 8 (33) | 31 (26) |
| Cardiac involvement | 18 (7.6) | 5 (56) | 3 (10) | 5 (9.3) | 2 (8.3) | 3 (2.5) |
| Digital ulcers | 70 (30) | 5 (56) | 11 (37) | 19 (35) | 7 (29) | 28 (24) |
Patient clinical characteristics overall and for each trajectory during the baseline period of 3 years from disease onset. Age of onset is described as median (IQR), and other categorical variables are described as number (%).
*Of the patients who were negative for all three autoantibodies shown, 15 were ANA positive (10 with the nucleolar pattern) and 19 were ANA negative.
ANA, antinuclear antibody; PAP, pulmonary arterial pressure; SSc, systemic sclerosis.