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

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.