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Rheumatology Advance Access published online on July 20, 2004
Rheumatology, doi:10.1093/rheumatology/keh326
Rheumatology © British Society for Rheumatology 2004; all rights reserved
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Received March 24, 2004
Accepted June 21, 2004

Grand rounds in rheumatology

Chronic periaortitis

R. N. Jois 1*, K. Gaffney 1, T. Marshall 1, D. G. I. Scott 1

1 Norfolk and Norwich University Hospital, Department of Rheumatology, Norwich, UK

* To whom correspondence should be addressed. E-mail: ramesh.jois{at}nnuh.nhs.uk.


  Abstract

Chronic periaortitis commonly involves the infrarenal portion of the abdominal aorta. Idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysm and perianeurysmal retroperitoneal fibrosis are its various clinical presentations. They present as a non-specific systemic inflammatory disorder and may lead to ureteric obstruction and consequent renal failure. An exaggerated inflammatory response to advanced atherosclerosis has been thought to be the main pathogenetic process. Autoimmunity has also been proposed as a contributing factor. Contrast-enhanced CT scanning is the diagnostic test of choice. Steroids and immunosuppressive agents are successfully used in the treatment of idiopathic retroperitoneal fibrosis and selected cases of inflammatory abdominal aortic aneurysm, and surgery is used in others. Early diagnosis is important in order to reduce morbidity from complications such as renal failure and mortality from aortic rupture.

Keywords: Chronic periaortitis; Idiopathic retroperitoneal fibrosis; Inflammatory aneurysm of abdominal aorta.
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