Table 1

Characteristics of the 60 patients with systemic sclerosis included in the discovery cohort

Age (years), median (range)

53.5 (28-81)

Disease duration (years), median (range)

9 (1-50)

Limited/Diffuse cutaneous subset, n (%)

36 (60)/24 (40)

Modified Rodnan skin score > 14, n (%)

19 (32)

History of digital ulcers, n (%)

29 (47)

Pulmonary fibrosis on CT scan, n (%)

28 (47)

Pulmonary arterial hypertension on RHC, n (%)

4 (7)

Positive antinuclear antibodies (> 1/160), n (%)

53 (88)

Positive antitopoisomerase-1 antibodies, n (%)

28 (47)

Positive anticentromere antibodies, n (%)

12 (20)

FVC < 75% predicted, n (%)

13 (22)

DLCO/AV < 75% predicted, n (%)

23 (38)

Treatment with calcium channel blockers, n (%)

60 (100)

Treatment with angiotensin-converting enzyme inhibitors, n (%)

19 (32)

Treatment with endothelin-receptor antagonists and/or PDE5 inhibitors, n (%)

12 (20)


DLCO/AV, diffusing capacity for carbon monoxide/alveolar volume; FVC, forced vital capacity; PDE5, phosphodiesterase type 5; RHC, right heart catheterization.

Avouac et al. Arthritis Research & Therapy 2013 15:R55   doi:10.1186/ar4217

Open Data