Patients' characteristics
| Age* (years) | Sex (n) | Disease duration† (years) | Disease subsets (n) | Extent of skin fibrosis assessed by the modified Rodnan Skin Score† | Antibody profile (n) | Major organ involvement except lung fibrosis‡ (n) |
|---|---|---|---|---|---|---|
| 54.0±13.0 | Men (n=6) | 2(0,64) | lSSc (n=21) | 9(2, 18)/51 | ANA positive (n=45) | Renal crisis (n=2) |
| Women (n=39) | dSSc (n=17) | ACA positive (n=15) | Heart (n=18) | |||
| Early SSc without skin fibrosis (n=5) | Scl70 positive (n=19) | Gastrointestinal tract (n=28) | ||||
| Overlap syndrome (n=2) | RNA-polymerase III positive ((n=3) (data from 22 patients are missing)) | Pulmonary hypertension (n=8) |
Heart involvement, palpitations reported by patients and conduction blocks, as assessed by ECG; diastolic dysfunction/pericardial effusion, as analysed by echocardiography; involvement of the gastrointestinal tract, dysphagia, reflux, early satiety, vomiting, diarrhoea, bloating and constipation, all defined according to the EUSTAR criteria;12 pulmonary hypertension, as assessed by right heart catheterisation according to the Dana point criteria 2008;13 mPAP >25mm Hg at rest, pulmonary wedge pressure ≤15 mm Hg,
SSc according to the criteria by LeRoy 1988;19 early SSc without skin fibrosis, according to the criteria by LeRoy and Medsger 2001;20 overlap syndromes: one patient with SSc and polymyositis overlap, one patient with SSc and rheumatoid arthritis overlap.
↵* numbers are expressed by mean±SD.
↵† numbers are given by median(Q1,Q3).
‡ renal crisis, arterial hypertension and renal failure.
ACA, anti-centromer antibodies; ANA, anti-nuclear antibodies; dSSc, diffuse cutaneous SSc; lSSc, limited cutaneous SSc.