Total | dcSSc | lcSSc | |
Subjects n | 105 | 45 | 60 |
Age yrs | 52.7±11.8 | 52.2±13.4 | 53.1±10.6 |
Female | 90 (85.7) | 33 (73.3) | 57 (95.0) |
Black | 8 (7.6) | 5 (10.9) | 3 (5.1) |
SSc duration yrs# | 2.0 (0.0–5.0) | 2.0 (1.0–5.0) | 1.0 (0.0–4.0) |
Prior digital ulcer history | 32 (30.5) | 18 (39.1) | 14 (23.7) |
PAH¶ | 1 (0.9) | 1 (2.2) | 0 (0) |
Medsger stage ≥3+ | 15 (14.4) | 8 (17.4) | 7 (11.9) |
ANA-positive§ | 89 (84.8) | 40 (88.9) | 49 (81.7) |
Anti-topoisomerase I | 30 (28.5) | 30 (65.2) | 0 (0) |
Anticentromere antibody | 21 (21.0) | 0 (0) | 21 (35.6) |
Anti-ribonucleoprotein | 8 (7.6) | 3 (6.7) | 5 (8.3) |
FVC <80% pred | 23 (22.9) | 15 (33.3) | 8 (13.3) |
FVC <70% pred | 7 (6.7) | 5 (10.9) | 2 (3.4) |
FVC <50% pred | 0 (0) | 0 (0) | 0 (0) |
DL,CO <80% predƒ | 60 (58.8) | 34 (73.9) | 26 (44.1) |
Abn oesophageal manometry | 80 (76.2) | 40 (88.9) | 40 (66.7) |
Hurwitz stage ≥3+ | 57 (54.6) | 30 (65.2) | 27 (45.8) |
Oesophageal aperistalsis | 38 (36.2) | 21 (45.6) | 17 (28.8) |
LOS pressure <10 mmHg | 52 (49.5) | 26 (57.7) | 26 (43.3) |
Data are presented as mean±sd, n (%) or median (interquartile range), unless otherwise indicated. dcSSc: diffuse cutaneous SSc; lcSSc: limited cutanous SSc; PAH: pulmonary arterial hypertension; ANA: antinuclear antibody; FVC: forced vital capacity; % pred: % predicted; DL,CO: diffusing capacity of the lung for carbon monoxide; Abn: abnormal; LOS: lower oesophageal sphincter. #: from diagnosis of first non-Raynaud symptom; ¶: pre-capillary PAH diagnosed by right heart catheterisation (n = 101); +: n = 104, diagnosed by histology; §: dilution of more than 1:80; ƒ: n = 102.