Open AccessEfficacy of immunosuppressants with bridge vasodilator therapy in severe lupus erythematosus ‐associated pulmonary arterial hypertension
Author(s) -
Sanges Sébastien,
Savale Laurent,
Lamblin Nicolas,
RémyJardin Martine,
Humbert Marc,
Sobanski Vincent
Publication year - 2019
Publication title -
esc heart failure
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.787
H-Index - 25
ISSN - 2055-5822
DOI - 10.1002/ehf2.12507
Subject(s) - medicine , bosentan , tadalafil , cardiogenic shock , cardiology , pulmonary hypertension , population , cyclophosphamide , sildenafil , chemotherapy , endothelin receptor , receptor , environmental health , myocardial infarction
Optimal management of systemic lupus erythematosus (SLE)‐associated pulmonary arterial hypertension (PAH) remains unclear. Our observation describes the case of a 31‐year‐old SLE patient presenting with cardiogenic shock revealing severe PAH, in which a therapeutic scheme combining immunosuppressants (pulse cyclophosphamide and corticosteroids) and PAH‐specific drugs (bosentan, tadalafil, and epoprostenol) led to a complete normalization of pulmonary haemodynamics and allowed a progressive weaning of PAH vasodilators. This case report supports the efficacy of immunosuppressants and use of PAH‐specific therapy as a bridge therapy in severe SLE‐PAH. Further studies on larger population are required to confirm these findings.