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.