Treatment of pre-capillary SAPH
| Highest level of evidence study in patients with SAPH | Total number of patients with SAPH treated | Results in sarcoidosis | |
| Prostenoids | |||
| Epoprostenol | Retrospective OL positive [78, 79] | 12 | Haemodynamics improved [78, 79] |
| Iloprost | Prospective, OL [72] | 15 of 22 enrolled completed 16 weeks’ therapy | In sarcoidosis, haemodynamics and QoL improved [72] |
| Endothelin receptor antagonists | |||
| Bosentan | DBPC [71] | 23 | Haemodynamics improved, no change in 6MWD [71] |
| Ambrisentan | Prospective OL [73] | 21 | Nonsignificant improved QoL, no change 6MWD [73] |
| Macitentan | Retrospective OL [82] | 6 | WHO FC improved in 4/6 treated patients [82] |
| Phosphodiesterase 5 inhibitors | |||
| Sildenafil | Retrospective OL [16] | 12 | Haemodynamics improved, 6MWD no changes |
| Tadalafil | Prospective OL [74] | 12 | No significant changes in 6MWD and QoL |
| Others | |||
| Riociguat | DBPC [70] | 16 | TCW and 6MWD significantly better compared with placebo |
| Combination therapy | Retrospective OL positive [8, 21, 77] | 29 | Haemodynamics and 6MWD improved in some |
SAPH: sarcoidosis-associated pulmonary hypertension; OL: open-label; QoL: quality of life; DBPC: double-blind, placebo-controlled; 6MWD: 6-min walk distance; WHO FC: World Health Organization functional class; TCW: time to clinical worsening.