Scleroderma renal crisis complicated by hemolytic uremic syndrome in a case of elderly onset systemic sclerosis

We report a case of renal crisis in a patient with elderly onset systemic scleroderma (SSc). A sixty-one-year-old woman was diagnosed as having SSc with rapidly advancing generalized skin sclerosis. After experiencing an upper respiratory infection, she suddenly developed renal failure, hemolytic anemia and malignant hypertension. Laboratory examination revealed uremia with a significantly high plasma renin level. Ophthalmologic study revealed Keith-Wagner's retinopathy Grade IV. Combination therapy including captopril, systemic corticosteroid and prostaglandin E1 venous infusion for the hemolytic uremic syndrome was effective and saved her from the renal crisis. However, her renal function deteriorated and she needs permanent hemodialysis. Rapidly progressive skin sclerosis in SSc, especially in elderly onset cases, suggests a high risk for renal crisis and indicates the need for careful consideration of hypertension and renal function.