Table 1

Baseline clinical characteristics of the 236 patients with SSc

Clinical factorsAll
(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)
Female204 (86)8 (89)27 (90)42 (78)22 (92)105 (88)
Ever smoking65 (28)4 (44)9 (30)16 (30)6 (25)30 (25)
Hypertension81 (34)5 (56)13 (43)24 (44)8 (33)31 (26)
Type of autoantibody*
 Anti-centromere142 (60)4 (44)13 (43)38 (70)12 (50)75 (63)
 Anti-topoisomerase I47 (20)1 (11)7 (23)12 (22)7 (29)20 (17)
 Anti-RNA polymerase III13 (5.5)2 (22)3 (10)1 (1.9)1 (4.2)6 (5.0)
Diffuse cutaneous SSc58 (25)2 (22)13 (43)14 (26)8 (33)21 (18)
Lung fibrosis82 (35)6 (67)16 (53)21 (39)8 (33)31 (26)
Cardiac involvement18 (7.6)5 (56)3 (10)5 (9.3)2 (8.3)3 (2.5)
Digital ulcers70 (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.