Retroperitoneal fibrosis

Augusto Vaglio, Carlo Salvarani, Carlo Buzio

Research output: Contribution to journalArticle

Abstract

Retroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighbouring structures - eg, ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings - namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants - and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease. Steroids are normally used to treat idiopathic retroperitoneal fibrosis, although other options - eg, immunosuppressants, tamoxifen - are available. The outlook is usually good, but, if not appropriately diagnosed or treated, the disease can cause severe complications, such as end-stage renal failure. Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms.

Original languageEnglish
Pages (from-to)241-251
Number of pages11
JournalLancet
Volume367
Issue number9506
DOIs
Publication statusPublished - Jan 21 2006

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Vaglio, A., Salvarani, C., & Buzio, C. (2006). Retroperitoneal fibrosis. Lancet, 367(9506), 241-251. https://doi.org/10.1016/S0140-6736(06)68035-5