The effects of the converting enzyme inhibitor captopril (Lopril) were studied in a 53 year old woman with acute exacerbation of scleroderma. In addition to her chronic symptoms of Raynaud's syndrome, the patient presented with severe hypertension, cardiac failure and oligoanuria. Right heart catheterisation with a Swan-Ganz catheter confirmed the systemic hypertension with cardiac failure, and also demonstrated precapillary pulmonary hypertension with raised pulmonary arterial resistance. The organic renal failure was an indication for renal biopsy which showed segmental and focal fibrinoid necrosis with microthrombosis and chronic ischemic changes. Due to raised plasma renin activity, treatment with captopril was instituted, leading to a rapid normalisation of systemic and pulmonary hypertension, the regression of cardiac failure and a transient improvement in the Raynaud's syndrome. The renal failure did not improve and the patient had to undergo chronic hemodialysis. These spectacular initial results should be interpreted in the context of the poor prognosis of acute exacerbations of scleroderma despite the encouraging data published recently after well-controlled antihypertensive therapy.

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http://dx.doi.org/10.1016/s0248-8663(83)80004-6DOI Listing

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