Table 1.
Clinical and laboratory features of the study population. Abbreviations: CTD—connective tissue diseases; PAH—pulmonary arterial hypertension.
| Clinical Features | Study Population | CTD without PAH | CTD-PAH | p |
|---|---|---|---|---|
| Female gender | 101 (89.4) | 87 (89.7) | 14 (87.5) | 0.68 |
| Median age, years | 65.0 (54.0–75.0) | 62.0 (51.0–71.0) | 74.0 (69.0–78.5) | 0.0004 |
| Hydroxychloroquine | 65 (57.7) | 57 (58.8) | 8 (50.0) | 0.59 |
| Methotrexate | 15 (13.3) | 15 (15.5) | 0 (0.0) | 0.12 |
| Steroids | 38 (33.6) | 36 (37.1) | 2 (12.5) | 0.08 |
| Phosphodiesterase 5 inhibitors | 6 (5.3) | 2 (2.1) | 6 (37.5) | <0.0001 |
| Endothelin-1 receptors antagonists | 8 (7.1) | 7 (7.2) | 8 (50.0) | <0.0001 |
| Riociguat | 1 (0.9) | 0 (0.0) | 1 (6.2) | 0.14 |
| Raynaud’s phenomenon | 102 (90.3) | 87 (89.7) | 14 (87.5) | 0.61 |
| Previous acral ulcers | 49 (43.4) | 43 (44.3) | 7 (43.7) | 1.00 |
| Digital ulcers in the past month | 5 (4.4) | 4 (4.1) | 1 (6.2) | 0.54 |
| Sclerodactyly | 66 (58.4) | 57 (58.8) | 9 (56.2) | 1.00 |
| Puffy fingers | 16 (14.2) | 16 (16.5) | 0 (0.0) | 0.12 |
| Telangiectasia | 32 (28.3) | 28 (28.9) | 5 (31.2) | 1.00 |
| Pulmonary interstitial disease | 38 (33.6) | 31 (32.0) | 7 (43.7) | 0.40 |
| Gastrointestinal involvement | 22 (19.5) | 18 (18.6) | 4 (25.0) | 0.51 |
| Renal involvement | 3 (2.7) | 2 (2.1) | 1 (6.2) | 0.37 |
| Anti-nuclear antibodies (ANA) | 104 (92.0) | 88 (90.7) | 16 (100.0) | 0.35 |
| Anti-centromere antibodies | 69 (61.1) | 56 (57.7) | 13 (81.2) | 0.10 |
| Anti-Scl-70 antibodies | 28 (24.8) | 23 (23.7) | 5 (31.2) | 0.54 |
| Anti-U1-RNP antibodies | 21 (18.6) | 16 (16.5) | 5 (31.2) | 0.17 |
| Disease duration | 5 (3–13) | 5 (4–13) | 5 (3–11) | 0.66 |