Follow-up of vasoactive therapy effect
| Patient | Therapy at diagnosis | WHO-FC at PH diagnosis | Therapy adjustments | Observed follow-up (months) | Description outcome | WHO-FC at end follow-up | Final outcome |
| 1 | S | 2 | S↑: 18 months S↑: 36 months | 36 | Slow progression to WHO-FC 3 | 3 | Alive |
| 2 | S | 3 | S + I: 12 months | 16 | Remains at WHO-FC 3 after iloprost therapy | 3 | Alive |
| 3 | B | 2 | B + S: 7 months B + S + T: 24 months | 36 | Slow progression to WHO-FC 3 6-MWT at diagnosis 420 m, at 8 months 459 m, at 28 months 390m | 4 | Death due to heart failure after 36 months |
| 6 | B | 3 | B↑: 48 months | 48 | With bosentan no PH after 12 months, after 48 months progressive dyspnoea | NA | Alive |
| 7 | S | 2 | Stop S: 8 months | 24 | Exacerbation of PM after 8 months, ↑ prednisone and start tacrolimus | 4 | Death due to respiratory failure after 24 months |
| 8 | S | 3 | NA | 0 | Unknown due to transfer to other hospital | NA | NA |
| 9 | A | 3 | Diuretics: 2 months | 12 | Short hospitalisation after 2 months due to heart failure, after 12 months stable in WHO-FC 3 | 3 | Alive |
↵A, ambrisentan; B, bosentan; I, iloprost inhalation; S, sildenafil; T, treprostinil; ↑, increase of dose; WHO-FC, WHO functional class; 6-MWT, 6 min walking test; PM, polymyositis; NA, not available.