RENAL INVOLVEMENT IN PROGRESSIVE SYSTEMIC SCLEROSIS (SCLERODERMA)

Abstract
Renal disease is a not uncommon manifestation of progressive systemic sclerosis. Although this renal involvement may run a benign, protracted course, the more common and significant manifestation of this lesion is an acute renal failure, with relentlessly progressive oliguria and azotemia, often associated with a malignant form of hypertension. In this situation a characteristic triad of lesions is observed in the kidney (1) intimal proliferation of the small intralobular arteries and arterioles; (2) fibrinoid necrosis involving the walls of the afferent arterioles and sometimes the glomerular loops; (3) focal cortical infarctions. The course when this acute renal lesion becomes clinically manifest is unremittingly downhill. Currently available modes of therapy are not apparently effective, and may even be deleterious. Five illustrative cases, of which 4 were autopsied, are presented.

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