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HLA-B27 (antigen) in retroperitoneal fibrosis in a family

Author(s): Mohammad Yazdani | Afshin Shadmehr

Journal: Archives of Medical Science
ISSN 1734-1922

Volume: 3;
Issue: 1;
Start page: 76;
Date: 2007;
Original page

Keywords: retroperitoneal fibrosis | HLA-B27 | --- familial retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis is a rare disease of undetermined aetiology. It is important to distinguish this entity from retroperitoneal fibrosis secondary to malignancy or specific inflammatory disease. There have been no prior means of excluding this condition without surgical exploration and histopathologic study of the excised tissue. A genetic predisposition is suggested for the development of idiopathic primary retroperitoneal fibrosis in patients who are HLA-B27 antigen positive. In this study we present three cases of idiopathic retroperitoneal fibrosis in a family (2 brothers and their grandfather). The presence of HLA-B27 antigen positivity was identified in two of them.

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