Normotensive renal failure in systemic sclerosis

Systemic sclerosis, also known as scleroderma, is a rare autoimmune disease affecting connective tissue and characterized by thickening of the skin due to deposits of collagen, a fibrous protein. One of the most serious complications resulting from scleroderma is kidney or renal failure, which usually occurs in patients who also have high blood pressure or hypertension. However, a number of studies describe scleroderma patients having renal failure without hypertension. Over a 33 year period 131 patients who experienced scleroderma related kidney failure were followed. Eleven percent of this group had normal blood pressure or were normotensive. Researchers attempted to isolate factors that would cause a normotensive scleroderma patient to develop renal failure. Their analysis revealed that these patients had significantly higher incidences than the hypertensive patients of various diseases: asthma (80 percent versus 33 percent of hypertensives); small blood vessel diseases (90 percent versus 38 percent); and an abnormal decrease in blood platelets or thrombocytopenia (83 percent versus 21 percent). Another significant finding in the medical histories of the normotensive scleroderma patients who developed renal failure was the fact that 64 percent of them had been administered maximum doses of corticosteroids during the two months prior to the onset of kidney failure, compared to only 16 percent of the hypertensive scleroderma patients. Since both scleroderma and corticosteroid medications can cause injury to circulatory tissue or vascular endothelium, it seems likely that high doses of corticosteroids were a contributing factor to the onset of kidney failure. It is recommended that only low doses of corticosteroids be prescribed for patients with scleroderma who are at high risk for developing kidney failure.