Successful treatment of systemic lupus erythematosus and pulmonary hypertension with intravenous prostaglandin I2 followed by its oral analogue

Abstract

Systemic lupus erythematosus (SLE) is sometimes reported to complicate fatal pulmonary hypertension. A 46-year-old woman, with a ten-year history of SLE and pulmonary hypertension, was admitted to our hospital complaining of dyspnea and chest pain. She suffered pulmonary hemorrhage and after steroid pulse therapy, she underwent continuous intravenous infusion of epoprostenol (prostaglandin I2) with corticosteroid for four weeks, which reduced the pulmonary artery pressure and resistance. Following the successful treatment, beraprost sodium, an oral PGI2 analogue, was given and it maintained pulmonary hypertension remittance for four years.