A 32-year-old African American woman, AW, presented with severe hypertension and acute renal failure. The patient had a four-year history of labile hypertension but was currently without medication. She described a 40-lb weight loss over the previous six months and for two months she had persistent anorexia, nausea, and fatigue.Admission physical examination was remarkable for a blood pressure of 210/160. Funduscopic examination showed a single left retinal flame hemorrhage and bilateral diffuse arteriolar narrowing. Additionally, she had periorbital edema and a faint rash on her eyelids. She had palpable hepatomegaly and sclerodactyly of her fingers. The remainder of her examination was normal.Admission laboratory revealed a blood urea nitrogen of 36 mgldL, a serum creatinine of 4.2 mg/dL, and mildly elevated liver function tests. Urinalysis showed microscopic hematuria and granular casts. A renal sonogram showed normal-sized kidneys with increased echogenicity.Over several days, the patient's serum creatinine rose to 10.0 mg/dL. Elective percutaneous renal biopsy revealed normal glomeruli and fibrinoid necrosis of the renal arterioles, consistent with malignant hypertension. Additional laboratory evaluation was remarkable for a positive antinuclear antibody (ANA) with a titer greater than 1:4,096 in a nucleolar pattern.Anti-DNA antibodies were negative and the C3, C4, CH.50, and serum protein electrophoresis were normal. A presumptive diagnosis of malignant hypertension secondary to scleroderma renal crisis was considered.Treatment consisted of strict blood pressure control using oral captopril and clonidine. Progressively, she became uremic and hemodialysis was initiated. A permanent vascular access