Resolution of renal failure with malignant hypertension in scleroderma: Case report and review of the literature

Abstract

The ominous prognosis of rapidly progressive renal failure associated with malignant hypertension in scleroderma has led to aggressive management by dialysis, early bilateral nephrectomy and renal transplantation. We describe a woman with scleroderma who recovered after the development of malignant hypertension and renal failure. Renal biopsy and arteriography demonstrated the classic vascular lesions of scleroderma with secondary cortical ischemia. Of note, plasma renin activity was normal. Review of the literature revealed 40 patients with scleroderma and rapidly progressive renal failure who have been treated by dialysis. Thirteen patients survived, four of whom did not undergo bilateral nephrectomy. In eight patients treated by transplantation, five achieved excellent allograft function although one sustained a late rejection. Analysis of our case and of five recently reported cases of reversible renal failure and malignant hypertension reveals no distinctive features identifying patients with a favorable prognosis, except for the normal plasma renin activity level in our patient. Vigorous control of hypertension and renal failure by drugs and dialysis is recommended. Bilateral nephrectomy should be considered only for patients with refractory hypertension, since recovery of renal function may follow even after months of dialysis. For patients with irreversible renal failure without other major organ system involvement, transplantation is a reasonable alternative to dialysis.