Resolution of scleroderma renal crisis with thrombotic microangiopathy. Case report (author's transl)

Renal involvement is a common cause of death in patients with systemic sclerosis (diffuse scleroderma). The poor prognosis is due to malignant hypertension and rapid deterioration of renal function. Therefore bilateral nephrectomy followed by hemodialysis and transplantation are proposed to avoid fatal outcome. The authors describe the case of a 37 year-old male suffering from diffuse scleroderma, and who developed malignant hypertension and renal failure. The renal biopsy and biological investigations showed thrombotic microangiopathy. The plasma renin activity was in a very high range. Control of hypertension and improvement of renal function were obtained by major antihypertensive drugs, i.e. intra-muscular clonidine, propranolol and dihydralazine. 14 months later, plasma creatinine is 2 mg/100 ml and blood pressure is 140/80 mm Hg. This case and similar ones reviewed in the literature demonstrate that bilateral nephrectomy should be performed in "scleroderma renal crisis" only after failure of very potent antihypertensive therapy.