Renal vascular histology and morphometry in systemic sclerosis. a case–control autopsy study

Abstract

An autopsy case-control study of renal vascular histology and morphometry in systemic sclerosis (scleroderma) was performed. Thirty-five of 70 systemic sclerosis cases had renal tissue available for study: 26 had diffuse cutaneous involvement (9 with "renal crisis" and 17 without) and 9 had limited cutaneous disease (CREST syndrome [calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias]).Age-matched (within 10 years) and sex-matched controls with renal specimens were obtained. New sections were cut from tissue blocks, and morphometry was completed using a Zeiss Image Analyzer. Using analysis of variance, the intimal area (Ai) was significantly increased (intimal thickening) in small and mediumsized arteries of patients with diffuse scleroderma and in small arteries of CREST patients, compared with those in controls, while a decreased medial area (Am) was seen consistently in all groups. The proportion of the vessel wall occupied by intima (Ai:[Ai + Am]) was significantly greater in all vessel size groups in patients with diffuse scleroderma compared with that in con-

Prognostic Factors of Renal Involvement in Systemic Sclerosis

“…Notably, manifestations of renal disease are observed clinically in up to 50% of SSc patients [5], even if autopsy studies revealed histological evidence of renal impairment in up to 80% of these patients [6]. …”

Thrombotic Thrombocytopenic Purpura with Severe Hypertension in a Patient with Systemic Sclerosis Sine Scleroderma and Polymyositis

“…Chronic changes include reduplication of the glomerular basement membranes, intimal fibrosis with severe luminal narrowing in arteries and arterioles, organization and recanalization of luminal thrombi, interstitial fibrosis, and glomerulosclerosis as a result of chronic ischemic damages. Marked intimal thickening leads to severe luminal narrowing in renal arteries after SRC (9). However, it is difficult to determine the cause of intimal fibrosis without severe luminal narrowing, interstitial fibrosis and glomerulosclerosis, as in the present case, because this pattern of chronic changes may be seen in the chronic stage of TTP, longstanding essential hypertension, and SSc without SRC (9).…”

“…Marked intimal thickening leads to severe luminal narrowing in renal arteries after SRC (9). However, it is difficult to determine the cause of intimal fibrosis without severe luminal narrowing, interstitial fibrosis and glomerulosclerosis, as in the present case, because this pattern of chronic changes may be seen in the chronic stage of TTP, longstanding essential hypertension, and SSc without SRC (9).…”

N-acetylcysteine infusion reduces the resistance index of renal artery in the early stage of systemic sclerosis

“…Although the pathogenesis of scleroderma renal crisis is unknown, endothelial cell injury, leading to narrowing and obliteration of the lumen of the vessels, is the primary pathogenetic mechanism. Vascular damage with arteries wall thickening is present in SSc patients without scleroderma renal crisis [29] . Renal vascular resistance was increased in main, interlobar and cortical vessels of SSc patients with normal renal function when compared with normal controls [30] .…”