Table 1

Description of the study cohort at baseline (total N=919)

Age (years)51 (40,60)
Male199/919 (21.7)
Female720/919 (78.3)
Disease duration (months)*42.5 (17,104)
Short disease duration ≤36 months*389/854 (45.6)
Raynaud's phenomenon898/919 (97.7)
Puffy fingers235/390 (60.3)
Digital ulcers (ever)396/914 (43.3)
Active digital ulcers84/394 (21.3)
mRSS16 (11,23)
Organ involvement
 Synovitis†196/915 (21.4)
 Joint contractures458/916 (50.0)
 Tendon friction rubs185/914 (20.2)
 Muscle weakness294/915 (32.1)
Dyspnoea (NYHA)
 Stage I210/369 (56.9)
 Stage II118/369 (32.0)
 Stage III37/369 (10.0)
 Stage IV4/369 (1.1)
 Conduction blocks109/877 (12.4)
 Diastolic dysfunction165/864 (19.1)
 LVEF <45%6/318 (1.9)
 Pulmonary hypertension by Echo167/868 (19.2)
 Lung fibrosis on chest X-ray389/849 (45.8)
 Lung fibrosis on HRCT163/287 (56.8)
 FVC <80%127/362 (35.1)
 TLC <80%80/245 (32.7)
 DLCO <70%357/621 (57.5)
Oesophageal symptoms625/917 (68.2)
Stomach symptoms257/916 (28.1)
Intestinal symptoms222/917 (24.2)
Renal crisis23/915 (2.5)
Laboratory markers
 ANA859/908 (94.6)
 ACA76/872 (8.7)
 Anti-Scl70524/886 (59.1)
 Anti-U1RNP16/285 (5.6)
 Anti-RNA polymerase III18/215 (8.4)
 CK elevation112/879 (12.7)
 Proteinuria74/887 (8.3)
 ESR>25 mm/1 h134/368 (36.4)
 CRP elevation107/374 (28.6)
 Active disease (VAI >3)12146/337 (43.3)
Immunosuppressive treatment334/436 (76.6)
  • For nominal variables, the absolute and relative frequencies are shown: n/total valid cases (%). Continuous variables are described as median and 1st, 3rd quartiles (Q1, Q3).

  • *Disease duration was calculated as difference between the date of the baseline visit and the date of the first non-Raynaud's symptom of the disease, as reported by the patients.

  • †Joint synovitis was defined as swelling of the joints as judged by the treating physician.

  • ACA, anticentromere antibodies; ANA, antinuclear antibodies; Anti-Scl70 antibodies, antitopoisomerase I antibodies; CK, creatine kinase; CRP, C reactive protein; DLCO, diffusing capacity of the lung for carbon monoxide; Echo, echocardiography; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; HRCT, high resolution computer tomography; LVEF, left ventricular ejection fraction; mRSS, modified Rodnan skin score; NYHA, New York Heart Association; RNP, ribonucleoprotein; TLC, total lung capacity; VAI, Valentini Activity Index.