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Table 1 Clinical and hemodynamic characteristics of the study population

From: Defining minimal detectable difference in echocardiographic measures of right ventricular function in systemic sclerosis

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
  1. Data are expressed as mean ± standard deviation for the continuous variables, and as absolute number and percentage for the discrete variables, as appropriate
  2. 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