Pulmonary arterial hypertension and fulminant myocarditis as initial manifestation of systemic lupus erythematosus

Abstract

Severe pulmonary arterial hypertension (PAH) is rarely observed as initial manifestation of systemic lupus erythematosus (SLE). Lupus myocarditis may rarely be initial presentation as a fatal complication of SLE, which mainly involved with the left ventricle. Here, we present a case of an incident PAH woman who had a delivery history 4 months previously. During hospitalization, she suffered from extensive myocardial damage, refractory ventricular tachycardia, and cardiogenic shock. Ultimately, she was diagnosed with SLE, fulminant lupus myocarditis, and PAH associated with connective tissue disease. After a large dose of methylprednisolone and PAH target therapy treatment, she recovered successfully and got a good result. To the best of our knowledge, this is the first case report of PAH and fulminant lupus myocarditis as initial manifestation of SLE in a previously healthy postpartum woman.

Keywords

• Myocarditis
• pulmonary arterial hypertension
• systemic lupus erythematosus