Table 1

Characteristics of the study population

Age (years)Sex (n)Disease duration* (months)Disease subsets (n)mRSS*Antibody profile (n of positives)Lung function parameters (n)Major organ involvement except ILD
56 (48,66)Men n=31/170
Women n=139/170
60 (28, 120)lcSSc (n=66/166)
dcSSc (n=34/166)
early/sine scleroderma (n=66/166)
lcSSc: 4 (2, 7)
dcSSc:13.5 (11, 19)
early/sine scleroderma 0 (0, 0)
ANA n=163/170
Scl-70 n=35/166
ACA n=73/166
RNA polymerase III n=11/160
U1-RNP n=7/164
Pm-Scl n=13/149
DLCO<70% of predicted n=43/168
FVC<70% of predicted n=11/168
TLC<70% of predicted n=13/168
Renal crisis n=3/167
GI involvement n=109/168
Heart involvement n=60/167
PAH n=7/170
  • Disease duration: time from first non-Raynaud symptom (missing data n=3).

  • GI involvement was defined as presence of symptoms of GI dysmotility.

  • Heart involvement was defined as presence of at least one of systolic/diastolic dysfunction on echocardiography, conduction block/significant arrhythmia on ECG.

  • PAH was defined as mPAP ≥25 mm Hg at rest and PWP≤15 mm Hg at RHC; RHC was performed when PH was suspected by the treating physician (29/170 patients).

  • *Median (Q1, Q3).

  • ACA, anticentromere antibodies; ANA, antinuclear antibodies; dcSSc, diffuse cutaneous SSc; DLCO, diffusing capacity of the lung for carbon monoxide; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; GI, gastrointestinal; ILD, interstitial lung disease; lcSSc, limited cutaneous SSc; mPAP, medium pulmonary artery pressure (RHC); PAH, pulmonary artery hypertension; PH, pulmonary hypertension; Pm-Scl, anti-Pm-Scl antibodies; PWP, pulmonary wedge pressure; RHC, right heart catheterisation; RNA polymerase III, anti-RNA polymerase III antibodies; Scl-70, antitopoisomerase 1 antibodies; SSc, systemic sclerosis; TLC, total lung capacity; U1-RNP, antiribonucleoprotein antibodies.