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We report a series of seven patients who had scleroderma renal crisis. Their primary clinical and laboratory features along with the details of their management were compared with those of similar cases from the literature. The seven patients died within one to four months of the diagnosis with a pattern of acute renal failure, left ventricular failure and malignant hypertension. Histopathologic examination was performed in four of the patients, in two of whom it revealed thickening of the wall of the interlobular arteries related to the scleroderma, and in the other two patients nonspecific lesions of malignant hypertension. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a lesion chronology of the kidney in patients with scleroderma. Nevertheless, the triggering factors and pathophysiologic mechanisms of scleroderma renal crisis remain unclear and its prognosis is severe. Early treatment with angiotensin-converting enzyme inhibitors and other vasodilatators administered intravenously can prevent death and dialysis.

140 cases of malignant hypertension were diagnosed in our clinic from January 1966 to December 1982. On admission the mean blood pressure was 183 +/- 17 mm Hg, and all patients had grade III to IV retinopathy according to the Keith and Wagener classification; 84% of the patients had renal failure (10% of acute origin). 43% of the patients presented with clinical signs of left heart failure. Hypertension was associated with various renal diseases in 48%, was essential in 41%, and renovascular lesions were found in 9% of the cases. Headaches, asthenia and visual disorders were the 3 main symptoms of malignant hypertension, as classically described. Severe cerebral damage (including all the neurological manifestations present on admission) was found in 27% of the patients. Among the 122 patients available to follow-up, half died during the study period. The survival rate, calculated on a 5-year basis, has doubled compared with a similar patient population 17 years ago, increasing from 35% (period 1966 to 1970) to 72% (period 1977-1982). This remarkable achievement in survival rate is due to more intensive research and therapeutic progress (including, more recently, extrarenal epuration) reaching an increasingly large hypertensive population.