Effectiveness and safety of endothelin receptor antagonists, alone and in combination therapy, in the pulmonary arterial hypertension-connective tissue disease subtype: A systematic review and meta-analysis.
Senthuran ShivakumarTilenka R ThynneLeila MohammadiChristine BurdeniukArduino Aleksander MangoniPublished in: International journal of rheumatic diseases (2020)
ERA, when used in combination with PDE5is, are associated with reduced risk of CFE, but no significant changes in 6MWD, in CTD-PAH. This warrants further studies investigating early combination therapy as a standard of care in this group.