2010
DOI: 10.1155/2010/543704
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Scleroderma Renal Crisis: A Pathology Perspective

Abstract: Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sc… Show more

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Cited by 95 publications
(110 citation statements)
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“…The histology displays proliferation and thickening of arcuate and interlobular arteriole intima, resulting in narrowing or full obliteration of vessels. There appears the typical picture of concentric (onion-like) hypertrophy 30 . The prognosis of this event is poor.…”
Section: Renal Crisismentioning
confidence: 99%
“…The histology displays proliferation and thickening of arcuate and interlobular arteriole intima, resulting in narrowing or full obliteration of vessels. There appears the typical picture of concentric (onion-like) hypertrophy 30 . The prognosis of this event is poor.…”
Section: Renal Crisismentioning
confidence: 99%
“…Vascular changes are accompanied by thromboses, the accumulation of myxoid material and the development of onion-skin lesions and fibrointimal sclerosis later in the disease course [29]. Vascular damage is a primary event in the pathogenesis of scleroderma.…”
Section: Connective Tissue Diseases and Sclerodermamentioning
confidence: 99%
“…Histologic correlate is presented by specific proliferation in the intima of arcuate and renal interlobular arteries and arterioles leading to narrowing or even obliteration of arteries with the formation of typical concentric hypertrophy ("onion skin") (ref. 10 ). Proliferative changes can be accompanied by arterial thrombosis and necrosis.…”
Section: Systemic Sclerodermamentioning
confidence: 99%