Table 2.
Key demographic and clinical data of SSc patients in the ELISA 1, Luminex and the ELISA 2.
| ELISA 1 (n = 297) | Luminex (n = 551) | ELISA 2 (n = 225) | |
|---|---|---|---|
| Age at disease onset, years | 48.7 (15.3) | 51.4 (15.4) | 52.2 (15.1) |
| Males, no (%) | 55 (18.5) | 93 (16.9) | 50 (22.2) |
| Diffuse cutaneous, no (%) | 78 (26.3) | 119 (21.6) | 51 (22.7) |
| Anti-centromere Ab, no (%) | 113 (38.0) | 284 (51.4) | 111 (50.0) |
| NYHA class, no (%) | 230 (57.9) | 496 (89.9) | 204 (90.7) |
| NYHA class 1-2, no (%) | 171 (43.1) | 403 (80.9) | 151 (74.0) |
| NYHA class 3-4, no (%) | 59 (14.8) | 93 (18.7) | 53 (26.0) |
| NT-pro BNP, pg/mL | 73.8 (304.9) | 86.5 (359.2) | 95.2 (350.6) |
| DLCO % | 68.2 (21.8) | 68.1 (20.0) | 65.1 (19.0) |
| Examined by ECHO, no (%) | 291 (97.9) | 457 (82.8) | 198 (88.0) |
| sPAP mmHg | 27.8 (19.2) | 27.0 (17.4) | 31.9 (21.4) |
| Examined by RHC, no (%) | 169 (56.9) | 251 (45.5) | 165 (73.3) |
| mPAP, mmHg | 28.9 (13.2) | 25.6 (11.1) | 28.8 (10.6) |
| PAWP, mmHg | 9.6 (7.3) | 9.5 (5.9) | 10.2 (6.6) |
| PVR, WU | 3.7 (4.0) | 3.7 (6.4) | 3.9 (7.6) |
| PAH, no (%) | 41 (24.3) | 72 (28.7) | 51 (22.7) |
| Time from disease onset to PH, years | 9.1 (8.8) | 8.1 (9.9) | 7.7 (9.9) |
| Time from serum sampling to PH, years | -0.33 (2.3) | -0.78 (3.1) | -0.84 (3.0) |
| Therapy, no (%) | 123 (41.4) | 284 (51.4) | 152 (67.6) |
| Immunosuppressive, no (%) | 50 (16.8) | 141 (25.5) | 76 (33.8) |
| PAH-treatment | 87 (29.3) | 156 (28.3) | 92 (40.9) |
| Mono therapy, no (%) | 42 (48.3) | 76 (48.7) | 43 (46.7) |
| Duo therapy, no (%) | 40 (45.9) | 67 (42.9) | 40 (43.5) |
| Triple therapy, no (%) | 5 (5.7) | 13 (8.3) | 9 (9.8) |
| Calcium channel blockers, no (%) | 35 (11.8) | 153 (27.7) | 78 (34.7) |
| ACE-inhibitors, no (%) | 13 (4.4) | 56 (10.1) | 33 (14.7) |
| Deceased, no (%) | 87 (29.3) | 126 (22.9) | 64 (28.4) |
The clinical and demographic data did not significantly differ in the OUH and USZ cohort, except from the percentage of deceased patients (22). Values are the mean (SD) if not indicated otherwise. NYHA, New York Heart Association; NT-pro BNP, N-terminal pro-Brain Natriuretic Peptide; DLCO, diffusing capacity of carbon monoxid; ECHO, Echocardiography; sPAP, systolic pulmonary artery pressure; RHC, right heart catheterization; PH, pulmonary hypertension; PAH, pulmonary arterial hypertension; mPAP, mean pulmonary artery pressure; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; Mono therapy, phosphodiesterase-5 inhibitors (PD5I) or endothelin receptor antagonists (ERA); Duo therapy, PD5I and ERA; Triple therapy, PD5I, ERA and prostacyclins (P); ACE-inhibitors, Angiotensin converting enzyme-inhibitors.