Abstract
A 59-year old Japanese woman diagnosed as having systemic sclerosis with limited cutaneous involvement was hospitalized with scleroderma renal crisis. Malignant hypertension with marked hyperreninemia and grade three hypertensive retinopathy were present, and the renal biopsy showed marked intimal thickening of the small intrarenal arteries. The patient's serum was negative for anticentromere and anti-DNA topoisomerase I (Scl-70) antibodies, whereas a radioimmunoprecipitation study revealed the presence of antibody to RNA polymerases I, II, and III. The patient was successfully treated with captopril, with normalization of blood pressure and reversal of the decreased renal function. The presence of antibody to RNA polymerases may be a marker for the development of life-threatening scleroderma renal crisis even in patients with limited cutaneous systemic sclerosis.
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