Successful treatment for pulmonary hypertension with angiotensin 1 converting enzyme inhibitor in a patient with mixed connective tissue disease

Abstract

This report described a 44 years-old female mixed connective tissue disease (MCTD) patient presenting pulmonary hypertension, successfully treated with angiotensin I converting enzyme (ACE) inhibitor. The patient was diagnosed as having MCTD because of Raynaud’ s phenomenon, swollen hand, and elevated level of anti-U 1 RNP antibodies. She was admitted to our hospital one year after the diagnosis of MCTD, because she experienced dyspnea and pretibial edema. A diagnosis of pulmonary hypertension was made by echocardiography and catheterization study. Pulmonary artery pressure and pulmonary artery resitance declined and returned gradually to normal during a period of three months with prednisolone and enarapril (10mg/day) therapy. Pulmonary hypertension is one of fetal complications with MCTD, and 5 year mortality rate is reported less than 50%. However, a successful therapy for the pulmonary hypertension with MCTD has not been established yet. We considered that ACE inhibitor might be useful to control the pulmonary hypertension with MCTD and could improve the prognosis of MCTD. © 1997, The Japan Society for Clinical Immunology. All rights reserved.