Clinical and haemodynamic characteristics of each patient at PH diagnosis
| Characteristics | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | Patient 9 |
| Clinical characteristics | PM | PM | PM | PM overlap SLE | DM | DM | PM | PM | PM |
| ANA | + | + | + | + | + | − | + | + | – |
| Anti-Jo1 | + | + | – | – | NA | + | + | – | + |
| Raynaud’s phenomenon | + | + | – | – | – | + | + | + | – |
| Arthritis (history) | + | + | + | – | + | + | + | + | – |
| Disease duration (years) | 20.4 | 8.8 | 5.1 | 1.2 | 5.8 | 0.6 | 10 | 5.4 | 10.7 |
| WHO functional class | 2 | 3 | 2 | 4 | 4 | 3 | 2 | 3 | 3 |
| WHO PH group | 3 | 3 | 1 | 2 | 1 | 3 | 3 | 3 | 1 |
| HRCT scan | |||||||||
| Lung fibrosis on HRCT | + | + | + | NA | – | + | + | + | + |
| Wells score | 2 | 3 | 2 | NA | 0 | 2 | 3 | 1 | 3 |
| Lung involvement (%) | 70 | 70 | 10 | NA | 0 | 90 | 30 | 90 | 10 |
| Extent of ILD | Extensive | Extensive | Limited | Extensive | Limited | Extensive | Extensive | Extensive | Limited |
| Pulmonary function test | |||||||||
| TLC (% of predicted) | 66 | 80 | 64 | 57 | 51 | 87 | 65 | 50 | 105 |
| DLCO (% of predicted) | 66 | 53 | 68 | NA | 39 | 67 | 69 | 62 | 42 |
| 6 Min walking test (m) | NA | 143 | 420 | NA | NA | NA | 308 | 235 | 307 |
| RHC | |||||||||
| Mean PAP (mm Hg) | 45 | 40 | 66 | 41 | 60 | 32 | 25 | 33 | 52 |
| PCWP (mm Hg) | 12 | 5 | 7 | 35 | 1 | 14 | 11 | 14 | 14 |
| Cardiac index (L/min/m2) | NA | NA | 2.3 | 1.4 | 2.2 | 2.6 | 2.5 | NA | 2.1 |
| Cardiac output (L/min) | NA | 3.8 | 5.1 | 3.1 | 2.7 | 4.6 | 4.6 | 3.3 | 3.3 |
| PVR (dynes/s/cm5) | NA | 743 | 928 | 155 | 1749 | 315 | 242 | 461 | 109 |
ANA, antinuclear antibody; DLCO, diffusing capacity for carbon monoxide; DM, dermatomyositis; ILD, interstitial lung disease; PAP, pulmonary arterial pressure; PCWP, pulmonary capillary wedge pressure; PH, pulmonary hypertension; PM, polymyositis; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; SLE, systemic lupus erythematosus; TLC, total lung capacity; NA, not available.