Chronic periaortitis: a spectrum of diseases

Our plain-English summary of Chronic periaortitis: a spectrum of diseases, by A. Vaglio, C. Buzio (Current Opinion in Rheumatology, 2005).

The study

• Authors: A. Vaglio, C. Buzio
• Journal: Current Opinion in Rheumatology
• Published: 2005;17(1):34-40
• Type of article: Review
• Cited by: 119 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Purpose Of Review: Chronic periaortitis includes idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis, and inflammatory abdominal aortic aneurysms. This review analyses the different aspects of the disease and highlights evolving concepts concerning its pathogenesis, diagnosis, and management.

Recent Findings: It has recently been reported that asbestos exposure is a major risk factor for idiopathic retroperitoneal fibrosis. An increasing number of studies showing an association with autoimmune diseases clearly support the hypothesis of a close link between autoimmunity and chronic periaortitis. Furthermore, various findings (eg, constitutional symptoms, the involvement of other organs, high acute-phase reactant levels) support the hypothesis that chronic periaortitis may be a manifestation of a systemic disease and challenge the well-known theory of a local immune response to antigens in atherosclerotic plaque. In addition to CT and MRI, which are the diagnostic modalities of choice, positron emission tomography may be useful in monitoring disease activity and response to therapy. Although there is a lack of prospective randomized trials, recent studies have highlighted the role of steroids, immunosuppressive agents, and tamoxifen in the medical treatment of chronic periaortitis.

Summary: Chronic periaortitis is a rare disease with protean manifestations but, if correctly diagnosed, can be successfully managed. It should be approached in the setting of a systemic process, and clinicians must be aware that other organs may be affected. Its clinical course is chronic-relapsing, so a careful follow-up is essential. Further studies are needed to investigate the pathogenetic mechanisms and the most appropriate therapeutic options.

In plain English

This article was a review, not an original study. The authors summarized published research on chronic periaortitis, a rare condition in which inflammatory, fibrous tissue forms around the abdominal aorta. The term covers idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. On asbestos, the review cited one case-control study (Uibu and colleagues, Lancet 2004). That study had reported that asbestos exposure was associated with a markedly increased risk of the disease. The review gave no fiber types, exposure levels or risk figures for asbestos. The same cited reference was the source of a Finnish annual incidence figure of 0.1 per 100,000 people. Smoking was also described as a significant risk factor. The review discussed links with autoimmune diseases, named CT and MRI as the main diagnostic tools, and described steroids as the mainstay of treatment. The authors concluded that the disease, if correctly diagnosed, can be successfully managed and should be approached as a systemic process. They noted a lack of prospective randomized trials.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative review of published literature on chronic periaortitis
• Who was studied: No study population of its own. The review covered published studies and case reports on chronic periaortitis (idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, perianeurysmal retroperitoneal fibrosis).
• Exposure: Asbestos exposure is mentioned only as a risk factor reported by a cited case-control study (Uibu et al., Lancet 2004). No fiber types, products, jobs or doses are given. Smoking and ergot derivatives are also mentioned as reported risk factors.
• Main results:
  • Cited study (Uibu et al., Lancet 2004): asbestos exposure was associated with a markedly increased risk of the disease. No risk estimate is given in the review.
  • Cited Finnish study (same reference): annual incidence of 0.1 per 100,000 people and prevalence of 1.38 per 100,000 inhabitants
  • Cited studies: smoking was also a significant risk factor
  • Cited case-control study: patients with inflammatory abdominal aortic aneurysms had a higher incidence of systemic autoimmune diseases than patients with noninflammatory aneurysms
  • CT and MRI are described as the main methods for diagnosis and follow-up
  • Steroids are described as the mainstay of medical treatment
• The authors' conclusion: The authors concluded that chronic periaortitis is a rare disease that, if correctly diagnosed, can be successfully managed, and that it should be approached as a systemic process.
• Limitations the authors state:
  • There is a lack of prospective randomized treatment trials
  • The epidemiology of chronic periaortitis is difficult to establish

Topics

Medical terms in this study, explained in our Lexicon: Idiopathic, Fibrosis, Asbestos Exposure.

Related studies

• Chronic Periaortitis: an Update — A. Palmisano, F. Maritati, et al. (2018)
• Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis — A. Vaglio (2009)
• IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review — S. Okamoto, H. Tsuboi, et al. (2020)
• Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study — M. Goldoni, S. Bonini, et al. (2014)
• Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation — E.F.H. van Bommel, I. Jansen, et al. (2009)
• Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure — V. Cottin, P.Y. Brillet, et al. (2008)

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