Renal changes in progressive systemic sclerosis

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IT IS well recognized that in patients with progressive systemic sclerosis (PSS) rapidly progressive renal failure may develop at any time during the course of the disease in association with severe hypertension, and result in death from uremia.1–5 The kidneys in those patients usually are described as having undergone two characteristic histologic changes: (1) intimal proliferation of interlobular arteries having a mucoid appearance, and (2) fibrinoid deposition in the intima or media of more distal interlobular arteries and afferent arterioles. These lesions comprise what is termed the “true scleroderma kidney.” Other changes that affect the glomeruli have received but limited interest. Our report presents five cases of PSS that demonstrated a variable clinical course and a spectrum of anatomic changes involving excretory as well as vascular structures of the renal cortex.

Specimens of renal tissue were obtained either by percutaneous needle biopsy with a modified Vim-Silvermann needle, under local anesthesia, or at autopsy. Specimens for light microscopy were divided into matched samples, half of which were fixed in Zenker’s solution, dehydrated, and embedded in paraffin. Six-micron-thick sections were stained with hematoxylin-eosin, Masson’s trichrome, PAS-alcian blue for mucopolysaccharides, Vcrhoeff’s stain for elastica, and Bowie’s stain for juxtaglomerular granules. The other half of the matched samples was sectioned in a cryostat after having been fixed in 4 percent phosphate buffered formalin and stained with Sudan IV or oil red O stains for lipids. For immunofluorescent studies, the renal tissue was frozen and sectioned (4 to 6 microns thick) on a cryostat. The . . .



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