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Case Reports
. 2007 Jan;26(1):115-6.
doi: 10.1007/s10067-005-0118-9. Epub 2005 Dec 20.

Retroperitoneal fibrosis and c-ANCA positivity

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Case Reports

Retroperitoneal fibrosis and c-ANCA positivity

Clio P Mavragani et al. Clin Rheumatol. 2007 Jan.

Abstract

Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease characterized by a chronic nonspecific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures. Although obscure, an autoimmune-mediated or vasculitic etiology has been hypothesized. In the current report, a case of RPF associated with the presence of antibodies against proteinase III, which responded effectively to immunosuppressive therapy, is presented.

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