Table 1—

Baseline demographic characteristics, pulmonary function test results and oesophageal profiles by systemic sclerosis(SSc) subtype

TotaldcSSclcSSc
Subjects n1054560
Age yrs52.7±11.852.2±13.453.1±10.6
Female90 (85.7)33 (73.3)57 (95.0)
Black8 (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 history32 (30.5)18 (39.1)14 (23.7)
PAH1 (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 I30 (28.5)30 (65.2)0 (0)
 Anticentromere antibody21 (21.0)0 (0)21 (35.6)
 Anti-ribonucleoprotein8 (7.6)3 (6.7)5 (8.3)
FVC <80% pred23 (22.9)15 (33.3)8 (13.3)
FVC <70% pred7 (6.7)5 (10.9)2 (3.4)
FVC <50% pred0 (0)0 (0)0 (0)
DL,CO <80% predƒ60 (58.8)34 (73.9)26 (44.1)
Abn oesophageal manometry80 (76.2)40 (88.9)40 (66.7)
Hurwitz stage ≥3+57 (54.6)30 (65.2)27 (45.8)
Oesophageal aperistalsis38 (36.2)21 (45.6)17 (28.8)
LOS pressure <10 mmHg52 (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.