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 | − |