A case of prostacyclin responsive pulmonary hypertension complicated in a patient with systemic lupus erythematosus.

A 36-year-old woman was admitted due to dyspnea and generalized edema, and was diagnosed as systemic lupus erythematosus and right ventricular heart failure due to accompanied pulmonary hypertension. The patient had been treated with diuretics, digoxin, high dose steroid and high dose nifedipine to treat right ventricular heart failure and pulmonary hypertension. Although the symptom and pulmonary hypertension responded transiently by these treatment, dyspnea was aggravated again and pulmonary arterial pressure was elevated in 1 week after high dose nifedipine treatment. The combination therapy of oral prostacyclin analogue (Beraprost), high dose steroid, azathioprine, hydrochloroquine sulfate was tried subsequently and decrease of pulmonary hypertension and improvement of clinical symptoms was noted in 45 days after beraprost treatment.