TABLE 1.
Patient characteristics at baseline
| Characteristic | PAH-CTD | PAH-SSc | PAH-SLE | PAH-MCTD/CTD-other | ||||
| Placebo | Selexipag | Placebo | Selexipag | Placebo | Selexipag | Placebo | Selexipag | |
| Patients n | 167 | 167 | 93 | 77 | 37 | 45 | 37 | 45 |
| Females | 146 (87.4) | 155 (92.8) | 76 (81.7) | 67 (87.0) | 36 (97.3) | 45 (100.0) | 34 (91.9) | 43 (95.6) |
| Age years | 52.8±15.0 | 51.8±14.1 | 61.2±9.9 | 58.6±11.2 | 38.6±11.3 | 39.3±11.4 | 46.1±15.0 | 52.5±12.6 |
| Geographic region | ||||||||
| Asia | 39 (23.4) | 48 (28.7) | 3 (3.2) | 7 (9.1) | 22 (59.5) | 26 (57.8) | 14 (37.8) | 15 (33.3) |
| Eastern Europe | 30 (18.0) | 28 (16.8) | 25 (26.9) | 16 (20.8) | 2 (5.4) | 4 (8.9) | 3 (8.1) | 8 (17.8) |
| Latin America | 12 (7.2) | 13 (7.8) | 6 (6.5) | 3 (3.9) | 3 (8.1) | 3 (6.7) | 3 (8.1) | 7 (15.6) |
| North America | 28 (16.8) | 33 (19.8) | 14 (15.1) | 21 (27.3) | 6 (16.2) | 6 (13.3) | 8 (21.6) | 6 (13.3) |
| Western Europe/Australia | 58 (34.7) | 45 (26.9) | 45 (48.4) | 30 (39.0) | 4 (10.8) | 6 (13.3) | 9 (24.3) | 9 (20.0) |
| Time since diagnosis of PAH years# | 1.7±2.3 | 1.6±2.3 | 1.6±2.1 | 1.5±2.2 | 1.7±2.2 | 1.4±1.9 | 2.1±2.8 | 2.0±2.8 |
| WHO functional class | ||||||||
| I | 3 (1.8) | 2 (2.6) | 1 (2.2) | |||||
| II | 74 (44.3) | 80 (47.9) | 35 (37.6) | 22 (28.6) | 24 (64.9) | 30 (66.7) | 15 (40.5) | 28 (62.2) |
| III | 92 (55.1) | 84 (50.3) | 57 (61.3) | 53 (68.8) | 13 (35.1) | 14 (31.1) | 22 (59.5) | 17 (37.8) |
| IV | 1 (0.6) | 1 (1.1) | ||||||
| 6-min walk distance m | 334.0±84.9 | 354.5±72.7 | 319.7±84.0 | 339.1±81.9 | 365.2±79.7 | 378.6±53.3 | 339.1±85.5 | 356.6±67.1 |
| Use of medication for PAH | ||||||||
| None | 42 (25.1) | 36 (21.6) | 25 (26.9) | 13 (16.9) | 10 (27.0) | 15 (33.3) | 7 (18.9) | 8 (17.8) |
| ERA | 26 (15.6) | 40 (24.0) | 12 (12.9) | 19 (24.7) | 8 (21.6) | 12 (26.7) | 6 (16.2) | 9 (20.0) |
| PDE-5i | 43 (25.7) | 51 (30.5) | 19 (20.4) | 20 (26.0) | 13 (35.1) | 9 (20.0) | 11 (29.7) | 22 (48.9) |
| ERA and PDE-5i | 56 (33.5) | 40 (24.0) | 37 (39.8) | 25 (32.5) | 6 (16.2) | 9 (20.0) | 13 (35.1) | 6 (13.3) |
| Other medications | ||||||||
| Immunosuppressants | 35 (21.0) | 28 (16.8) | 15 (16.1) | 10 (13.0) | 10 (27.0) | 9 (20.0) | 10 (27.0) | 9 (20.0) |
| Corticosteroids¶ | 81 (48.5) | 81 (48.5) | 30 (32.3) | 24 (31.2) | 26 (70.3) | 31 (68.9) | 25 (67.6) | 26 (57.8) |
| Calcium channel blockers | 45 (26.9) | 45 (26.9) | 30 (32.3) | 32 (41.6) | 7 (18.9) | 4 (8.9) | 8 (21.6) | 9 (20.0) |
| Cardiac therapy | 94 (56.3) | 95 (56.9) | 49 (52.7) | 39 (50.6) | 21 (56.8) | 26 (57.8) | 24 (64.9) | 30 (66.7) |
| Anti-hypertensives | 88 (52.7) | 89 (53.3) | 54 (58.1) | 51 (66.2) | 15 (40.5) | 22 (48.9) | 19 (51.4) | 16 (35.6) |
| Beta-blockers | 17 (10.2) | 12 (7.2) | 9 (9.7) | 7 (9.1) | 3 (8.1) | 1 (2.2) | 5 (13.5) | 4 (8.9) |
Data are presented as n (%) or mean±sd, unless otherwise stated. PAH-CTD: pulmonary arterial hypertension associated with connective tissue disease; PAH-SSc: PAH associated with systemic sclerosis; PAH-SLE: PAH associated with systemic lupus erythematosus; PAH-MCTD: PAH associated with mixed connective tissue disease; CTD: connective tissue disease; WHO: World Health Organization; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase type-5 inhibitor. #: confirmed by right heart catheterisation; ¶: for systemic use.