Malignant hypertension and uremia in scleroderma: efficacy of nephrectomy and hemodialysis

Two cases of scleroderma are presented in which malignant hypertension developed abruptly, accompanied by rapidly progressive renal failure. The malignant hypertension was associated with high plasma renin levels and like other forms of hyperrenninemic hypertension and uremia, was refractory to both antihypertensive medication and extracellular fluid volume control with hemodialysis. Blood pressures became controllable only after bilateral nephrectomy was performed, and in each case resulted in a reversal of a rapidly progressive downhill course. Though both patients had multisystem involvement at the onset of renal failure, the non-renal manifestations of scleroderma have not progressed rapidly. Consequently, when patients with scleroderma develop malignant hypertension and uremia, aggressive therapy with dialysis and nephrectomy may significantly prolong survival.