Chronic Periaortitis: an Update

Our plain-English summary of Chronic Periaortitis: an Update, by A. Palmisano, F. Maritati, A. Vaglio (Current Rheumatology Reports, 2018).

The study

• Authors: A. Palmisano, F. Maritati, A. Vaglio
• Journal: Current Rheumatology Reports
• Published: 2018;20(12):80
• Type of article: Review
• Cited by: 17 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Purpose Of Review: We aim to review traditional concepts and recent developments on the nosology, pathophysiology, clinical phenotypes and treatment of chronic periaortitis (CP).

Recent Findings: CP is a rare disorder hallmarked by a periaortic fibro-inflammatory tissue. It can present as an isolated disease, but it can also be associated with other autoimmune and fibro-inflammatory lesions (e.g., fibrosing mediastinitis, sclerosing pancreato-cholangitis) that are part of the spectrum of IgG4-related disease. In a subgroup of patients, it also involves the thoracic aorta (so-called "diffuse periaortitis"), which supports the notion of an inflammatory disorder of large arteries. The pathogenesis of CP is multifactorial: recent studies have elucidated the predisposing role of immunogenetic variants and exposures to environmental agents such as smoking and asbestos. CP is a rare immune-mediated disease that affects the abdominal aorta and the iliac arteries and, in some cases, the thoracic aorta. It may overlap with manifestations of IgG4-related disease, and its treatment comprises glucocorticoids, conventional and biological immunosuppressive agents.

In plain English

This article was a review, not a new study. The authors reviewed published work on chronic periaortitis. This is a rare disease in which inflamed, scar-like tissue forms around the abdominal aorta and the iliac arteries, often trapping the ureters and causing kidney blockage. The review covered how common the disease is, its symptoms, tissue findings, causes, diagnosis and treatment. On causes, the authors described several contributing factors, including immune-related gene variants and environmental exposures such as smoking and asbestos. They cited an earlier case-control study (Goldoni and colleagues, 2014) that linked idiopathic retroperitoneal fibrosis, one form of the disease, to asbestos exposure and to smoking. The review reported that the two exposures had a multiplicative effect in that study, with an odds ratio of 12.04 (95% confidence interval 4.32 to 38.28) in people exposed to both. The review gave no fiber types or exposure levels. It also reported that glucocorticoid treatment led to remission in about 75–95% of cases, and that relapse rates reached up to 72%. The authors concluded that the disease should be managed as a potentially systemic disease involving multiple organ systems.

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 new patients were studied. The review drew on published studies of chronic periaortitis, which includes idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis.
• Exposure: Asbestos exposure and smoking were discussed as environmental risk factors for the disease. No fiber types, products, jobs or doses were given.
• Main results:
  • The review cited a case-control study (reference 26, Goldoni et al. 2014). That study confirmed an association between asbestos exposure and the disease and identified smoking as a risk factor. Smoking and asbestos had a multiplicative effect, with an odds ratio of 12.04 (95% confidence interval, 4.32 to 38.28) in subjects exposed to both.
  • The review reported, from cited studies, that idiopathic retroperitoneal fibrosis has an incidence of 0.1–1.3 per 100,000 inhabitants per year and a prevalence of 1.4 per 100,000.
  • In the authors' own earlier study, thoracic vessel involvement was found in approximately 28 of the 77 chronic periaortitis patients studied (35%).
  • With glucocorticoids as first-line therapy, remission was reported in about 75–95% of cases.
  • Chronic periaortitis was described as a chronic-relapsing disorder, with relapse rates of up to 72%.
• The authors' conclusion: The authors concluded that chronic periaortitis is a fibro-inflammatory disease affecting large vessels. They wrote that it should be managed as a potentially systemic disease involving multiple organ systems, with autoimmune mechanisms likely to play a major role.
• Limitations the authors state:
  • The authors noted that evidence for the biologic drugs tocilizumab and rituximab is limited to small case series.
  • The authors stated that larger and prospective studies are needed to corroborate the findings on FDG-PET imaging and response to treatment.

Related studies

• Chronic periaortitis: a spectrum of diseases — A. Vaglio, C. Buzio (2005)
• Idiopathic retroperitoneal fibrosis: an update for nephrologists — V. Raglianti, G.M. Rossi, et al. (2021)
• Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis — A. Vaglio (2009)
• Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study — M. Goldoni, S. Bonini, et al. (2014)
• High serum immunoglobulin G4-related retrosternal fibrosclerosis — K. Kamiya, A. Yoshizu, et al. (2012)
• Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation — E.F.H. van Bommel, I. Jansen, et al. (2009)

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