Efficacy of immunosuppressants with bridge vasodilator therapy in severe lupus erythematosus ‐associated pulmonary arterial hypertension

AbstractOptimal management ofsystemic 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.

Source: ESC Heart Failure - September 18, 2019 Category: Cardiology Authors: S ébastien Sanges, Laurent Savale, Nicolas Lamblin, Martine Rémy‐Jardin, Marc Humbert, Vincent Sobanski Tags: Case Report Source Type: research