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Multifocal Fibrosclerosis Manifested by Vena Caval Obstructions and Associated with Vasculitis

Ann Intern Med. 1969;70(1):81-86. doi:10.7326/0003-4819-70-1-81
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Multifocal fibrosclerosis is the generic term currently use to describe a group of fibrosing conditions including chronic mediastinal fibrosis and retroperitoneal fibrosis. A 29-year-old brewery worker with obstructions of his superior and inferior vena cava due to mediastinal and retroperitoneal fibrosis was followed for 3 years until his death from hemoptysis. He also suffered from peripheral ulcerations due to disseminated vasculitis. Vasculitis has not been observed previously associated with chronic fibrous mediastinitis, although it has been found together with other members of the multifocal fibrosclerosis group (retroperitoneal fibrosis and sclerosing cholangitis).

It is hypothesized that inflammation of the blood vessels may lead to multifocal fibrosclerosis or may be present in association with this condition in response to the same primary stimulus.





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