SCLERODERMA RENAL CRISIS - 7 CASES AND A REVIEW OF THE LITERATURE

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 wit 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 fail ure and malignant hypertension. Histopathologic examination was perfor med 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 hypertensio n. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a l esion chronology of the kidney in patients with scleroderma. Neverthel ess, the triggering factors and pathophysiologic mechanisms of sclerod erma renal crisis remain unclear and its prognosis is severe. Early tr eatment with angiotensin-converting enzyme inhibitors and other vasodi latators administered intravenously can prevent death and dialysis.