Treatment of pre-capillary SAPH

Highest level of evidence study in patients with SAPHTotal number of patients with SAPH treatedResults in sarcoidosis
 EpoprostenolRetrospective OL positive [78, 79]12Haemodynamics improved [78, 79]
 IloprostProspective, OL [72]15 of 22 enrolled completed 16 weeks’ therapyIn sarcoidosis, haemodynamics and QoL improved [72]
Endothelin receptor antagonists
 BosentanDBPC [71]23Haemodynamics improved, no change in 6MWD [71]
 AmbrisentanProspective OL [73]21Nonsignificant improved QoL, no change 6MWD [73]
 MacitentanRetrospective OL [82]6WHO FC improved in 4/6 treated patients [82]
Phosphodiesterase 5 inhibitors
 SildenafilRetrospective OL [16]12Haemodynamics improved, 6MWD no changes
 TadalafilProspective OL [74]12No significant changes in 6MWD and QoL
 RiociguatDBPC [70]16TCW and 6MWD significantly better compared with placebo
 Combination therapyRetrospective OL positive [8, 21, 77]29Haemodynamics 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.