경구 prostacyclin 치료에 반응한 전신성 홍반성 낭창을 동반한 폐동맥 고혈압 1예
A case of prostacyclin responsive pulmonary hypertension complicated in a patient with systemic lupus erythematosus
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초록
전신성 홍반성 낭창에 동반된 폐동맥 고혈압은 매우 드물고 치료가 어렵다고 알려져 있다. 본 연구는 SLE와 동반된 폐동맥 고혈압 환자에서 고용량 칼슘 길항제의 경구 투여에서 효과가 없었으며 경구용 prostacyclin analogue, beraprost에 의하여 단기간의 폐동맥압 강압 효과와 임상 경과의 호전을 관찰하였으므로 보고하는 바이다.
Abstract
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.(Korean J Med 63:581-586, 2002)