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Idiopathic fibroelastosis of renal arteries and their relation to fibromuscular dysplasia

Virchows Archiv Abteilung A Pathologische Anatomie, ISSN: 0945-6317, Vol: 351, Issue: 2, Page: 99-121
1970
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  • Citations
    3
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      3

Article Description

Idiopathic fibroelastosis is a frequent lesion of renal arteries. Thereby, the inner vascular wall may become finely ondulated. Secondary adaptive fibrosis of the intima and in some instances lesions of the inner and outer elastic layers may occur. Aneurysma formation as a consequence of ruptures is found very rarely. Idiopathic fibroelastosis is the result of two main mechanisms: 1. degeneration of smooth muscle cells of the tunica media, 2. a change in functional properties of these muscle cells leading to an increased secretion of ground substance containing collagen and elastic fibers. The process cannot be related with alterations of high blood pressure, e.g. hypertension or low pressure due to stenosis of an ostium. Idiopathic fibroelastosis becomes manifest in the second decade of life and shows an increase in severity with age of the patient. The disease most likely has to be differentiated from fibromuscular dysplasia. Pathogenesis of idiopathic fibroelastosis is not yet clarified. The possibilities of either a primary insudation of constituents of blood plasm from the vascular lumen or a degeneration of the internal tunica elastica cannot be supported. The preferential involvement of kidney vessels suggests that repeated vascular spasms which are supposed to occur most frequently in renal vessels (e.g. shock) could be contributory in the alteration of muscle cells described. © 1970 Springer-Verlag.

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