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Clinical manifestations and diagnosis of retroperitoneal fibrosis

Augusto Vaglio, MD, PhD
Alessandra Palmisano, MD, PhD
Section Editor
Gary C Curhan, MD, ScD
Deputy Editor
Alice M Sheridan, MD


Retroperitoneal fibrosis is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs [1]. This disorder was initially called Ormond's disease [2], but it has also been referred to as periureteritis fibrosa, periureteritis plastica, chronic periureteritis, sclerosing retroperitoneal granuloma, and fibrous retroperitonitis.

Retroperitoneal fibrosis may be idiopathic or secondary to other causes. Idiopathic retroperitoneal fibrosis is an immune-mediated disease, which can be either isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibroinflammatory disorder, named immunoglobulin G4-related disease (IgG4-RD). Although there are no standardized criteria of classification, idiopathic retroperitoneal fibrosis is actually part of the disease spectrum of chronic periaortitis, a condition characterized by inflammation and fibrosis surrounding the aorta and iliac arteries [1,3,4]. Chronic periaortitis (CP) includes inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis, two entities that are grouped together because of similar clinical and histologic characteristics, although pathogeneses and epidemiology may differ [5]. In approximately one-third of patients, CP also involves the thoracic aorta and the epiaortic arteries, suggesting that it is a systemic inflammatory disease of the large arteries [6].

The etiology, pathogenesis, clinical manifestations, and diagnostic evaluation of retroperitoneal fibrosis will be reviewed here. The treatment of retroperitoneal fibrosis is presented separately (see "Treatment of retroperitoneal fibrosis"). Other causes of urinary tract obstruction and hydronephrosis are also reviewed elsewhere. (See "Clinical manifestations and diagnosis of urinary tract obstruction and hydronephrosis".)


Retroperitoneal fibrosis is a rare disease. In one Finnish study, the incidence of the idiopathic form of the disease was estimated from hospital discharge data to be 0.1 per 100,000 person-years and its prevalence 1.4 per 100,000 inhabitants [7]. However, in a subsequent population-based study performed in the Netherlands, the reported annual incidence was approximately 10-fold higher (ie, 1.3 per 100,000 inhabitants per year) [8]. The latter study probably provides a more accurate estimate of the incidence given the complexity of the diagnosis and requirement for prolonged follow-up that would not have been possible in a study of hospital discharge codes [9]. Inflammatory abdominal aortic aneurysms represent 4 to 10 percent of all abdominal aortic aneurysms [10]. No data are available about the incidence of secondary retroperitoneal fibrosis.

Idiopathic disease most commonly occurs in individuals 40 to 60 years of age [5,8,11,12]. Most studies have suggested a 2 to 3:1 male-to-female predominance [5,8,11,12], although this is not reported in all studies [4]. One study has suggested that chronic periaortitis, which includes idiopathic retroperitoneal fibrosis, is associated with the human leukocyte antigen (HLA) allele HLA-DRB1*03 [13].

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Literature review current through: Nov 2017. | This topic last updated: Sep 25, 2017.
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