Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis

Our plain-English summary of Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis, by A. Vaglio (Medicine, 2009).

The study

• Author: A. Vaglio
• Journal: Medicine
• Published: 2009;88(4):208-210
• Type of article: Comment
• Cited by: 24 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Summary of the article

This commentary discussed two prospective studies of idiopathic retroperitoneal fibrosis (RPF) by van Bommel et al and Scheel et al, published in the same issue of Medicine. The author noted that earlier RPF research had been limited by the disease's rarity, the lack of diagnostic and classification criteria, and the inclusion of heterogeneous patient groups. He reported that the population-based Dutch study gave an annual incidence of idiopathic RPF of 1.3 × 10^-5 inhabitants, more than 10-fold higher than an earlier Finnish estimate based on hospital discharge data. Pain was the most common symptom in both cohorts, affecting more than 90% of patients. Atherosclerotic disease was present in 40% and 20% of the two cohorts. In van Bommel's series, 20% of patients had been exposed to asbestos, most with pleural plaques. In Scheel's cohort, 12.5% had potential occupational asbestos exposure. The author considered it appropriate to classify RPF in asbestos-exposed patients as idiopathic rather than secondary, because it is indistinguishable radiologically and histologically and no clear causal relationship has been shown. He also discussed the typical imaging findings, Scheel's proposed four-class scheme based on disease extent, and limitations of both studies. He concluded that the studies extended knowledge of RPF and paved the way for future large controlled studies.

PubMed has no abstract for this study, and the article doesn't print one. We wrote this summary from the full article with the help of AI. It is not the authors' wording.

In plain English

This article was a short commentary, not an original study. The author discussed two studies of patients with idiopathic retroperitoneal fibrosis (RPF), published in the same journal issue by van Bommel et al and Scheel et al. As the author reported, 20% of patients in van Bommel's series had been exposed to asbestos, and most of them also had pleural plaques. In Scheel's cohort, 12.5% of patients had potential occupational exposure to asbestos. Citing a 2004 Finnish study, the author stated that asbestos exposure had been shown to increase the risk of RPF. The author nonetheless argued that RPF in asbestos-exposed patients should be grouped with idiopathic rather than secondary forms, because it looked the same on scans and in tissue as RPF in unexposed people and no clear causal relationship had been shown. The author also reported that van Bommel's study found an annual incidence of idiopathic RPF more than 10-fold higher than that reported in the Finnish study. The author noted that both studies described patients only at presentation, not over time.

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: commentary (editorial discussion) on two prospective cohort studies published in the same issue; no new data
• Who was studied: No patients were studied by the author. The commentary discussed two prospective cohorts of patients with idiopathic RPF. One was van Bommel et al, covering all RPF cases seen at the Albert Schweitzer Hospital, Dordrecht, The Netherlands, over 10 years. The other was Scheel et al. Patient numbers were not given in the commentary.
• Exposure: Asbestos exposure (fiber type not specified) was discussed as one of several possible risk or predisposing factors for idiopathic RPF, alongside atherosclerosis, certain drugs and radiation therapy. The commentary's main focus was clinical presentation and imaging.
• Main results:
  • In van Bommel's series (as reported by the author), 20% of patients had been exposed to asbestos, and most of them had pleural plaques.
  • In Scheel's cohort (as reported by the author), 12.5% of patients had potential occupational exposure to asbestos.
  • The author stated, citing a 2004 Finnish case-control study (reference 8), that asbestos exposure has been demonstrated to increase the risk of developing RPF.
  • Van Bommel's study found an annual incidence of idiopathic RPF more than 10-fold higher than the Finnish study's estimate. The exact figures are garbled by scanning in this text.
  • In both studies, more than 90% of patients presented with pain.
  • Atherosclerotic disease was present in up to 40% of patients in van Bommel's cohort and 20% in Scheel's cohort.
  • Both studies found that the typical scan appearance was periaortic, homogeneous tissue of muscle-like density that did not displace the aorta.
• The authors' conclusion: The author's view was that RPF in asbestos-exposed patients is appropriately classed among idiopathic, not secondary, forms. The reasons given were that it is indistinguishable on imaging and in tissue from RPF in non-exposed people, and that no clear causal relationship has been shown.
• Limitations the authors state:
  • The author stated that the two studies captured only presentation and could not show the course of the disease over time.
  • The author noted that because autoimmune diseases often appear late, their true frequency could not be established at RPF presentation.
  • The author noted that the Finnish incidence study identified cases from hospital discharge codes, a method that can be unreliable for idiopathic RPF.

Topics

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

Related studies

• 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)
• Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis — T. Uibu, R. Järvenpää, et al. (2008)
• Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study — M. Goldoni, S. Bonini, et al. (2014)
• Idiopathic retroperitoneal fibrosis: an update for nephrologists — V. Raglianti, G.M. Rossi, et al. (2021)
• Asbestos exposure as a risk factor for retroperitoneal fibrosis — T. Uibu, P. Oksa, et al. (2004)

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