Table 1.
Clinical and hemodynamic characteristics of the study population
| Characteristic | Total SSc population n = 20 | Cohort 1 SSc without PAH, n = 10 | Cohort 2 SSc with PAH n = 10 | P value |
|---|---|---|---|---|
| Age, years | 61.3 ± 8.2 | 60.9 ± 8.0 | 61.7 ± 8.9 | NS |
| Female sex, n (%) | 16 (80%) | 8 (80%) | 8 (80%) | NS |
| Disease subtype | ||||
| Limited, n (%) | 16 (80%) | 8 (80%) | 8 (80%) | NS |
| Diffuse, n (%) | 4 (20%) | 2 (20%) | 2 (20%) | NS |
| Autoantibody subtype, n (%) | ||||
| Positive anti-Scl70 | 2 (10%) | 0 | 2 (20%) | NS |
| Positive anti-centromere | 5 (25%) | 4 (40%) | 1 (10%) | NS |
| Positive anti-RNA polymerase | 0 | 0 | 0 | – |
| Positive ANA, centromere pattern | 8 (42.1%) | 5 (50%) | 3 (33%) | NS |
| Positive ANA, nucleolar pattern | 10 (52.6%) | 4 (40%) | 6 (67%) | NS |
| Positive ANA, RNP | 1 (5.3%) | 1 (10%) | 0 | NS |
| Medications (ever-usage) | ||||
| Calcium channel blockade, n (%) | 17 (85%) | 8 (80%) | 9 (90%) | NS |
| Azathioprine, n (%) | 0 | 0 | 0 | NS |
| Cychophosphamide, n (%) | 0 | 0 | 0 | − |
| D-Penicillamine, n (%) | 0 | 0 | 0 | − |
| Intravenous immunoglobulins, n (%) | 0 | 0 | 0 | − |
| Leflunomide, n (%) | 0 | 0 | 0 | − |
| Methotrexate, n (%) | 0 | 0 | 0 | − |
| Mycophenolate mofetil, n (%) | 5 (25%) | 1 (10%) | 4 (40%) | 0.04 |
| Plaquenil, n (%) | 6 (30%) | 4 (40%) | 2 (20%) | NS |
| Predisone, n (%) | 6 (31.6%) | 2 (22%) | 4 (40%) | NS |
| Rituximab, n (%) | 0 | 0 | 0 | − |
| PAH Therapies (ever-usage) | ||||
| Endothelin receptor antagonist, n (%) | 6 (31.6%) | 1 (11.1%) | 5 (50%) | NS |
| Phosphodiesterase type 5 inhibitors, n (%) | 9 (47.4%) | 1 (11.1%) | 8 (80%) | 0.001 |
| Prostacyclin analogs/receptor agonists, n (%) | 3 (15.8%) | 1 (11.1%) | 2 (20%) | NS |
| WHO FC I/II/III, n | 11/6/3 | 9/1/0 | 2/5/3 | 0.02 |
| FVC, % | 86 ± 21 | 92 ± 14 | 79 ± 26 | NS |
| 6-min walking distance, m | NA | NA | 397 ± 122 | − |
| Right atrial pressure, mmHg | NA | NA | 5 ± 4 | − |
| Mean PAP, mmHg | NA | NA | 36 ± 7 | − |
| PCWP, mmHg | NA | NA | 9 ± 4 | − |
| Cardiac Index, l/min/m2 | NA | NA | 2.3 ± 0.7 | − |
| PVR, WU | NA | NA | 6.1 ± 2.7 | − |
Data are expressed as mean ± standard deviation for the continuous variables, and as absolute number and percentage for the discrete variables, as appropriate
Abbreviations: SSc systemic sclerosis, PAH pulmonary arterial hypertension, ACRAmerican College of Rheumatology, WHO FC World Health Organization functional class, FVC forced vital capacity, NA not available or not applicable, PAP pulmonary arterial pressure, PCWP pulmonary capillary wedge pressure, PVR pulmonary vascular resistance, WU Wood unit