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

Identifieur interne : 000851 ( Main/Exploration ); précédent : 000850; suivant : 000852

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

Auteurs : Sébastien Sanges ; Laurent Savale ; Nicolas Lamblin ; Martine Rémy-Jardin ; Marc Humbert ; Vincent Sobanski

Source :

RBID : PMC:6989275

Abstract

Abstract

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.


Url:
DOI: 10.1002/ehf2.12507
PubMed: 31536678
PubMed Central: 6989275


Affiliations:


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