Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation

Our plain-English summary of Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation, by E.F.H. van Bommel, I. Jansen, T.R. Hendriksz, et al (Medicine, 2009).

The study

• Authors: E.F.H. van Bommel, I. Jansen, T.R. Hendriksz, A.L.H.J. Aarnoudse
• Journal: Medicine
• Published: 2009;88(4):193-201
• Cited by: 184 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Retroperitoneal fibrosis (RPF) is a rare disorder of unknown etiology. Its incidence is unknown, and the insidious and nonspecific nature of symptoms may contribute to considerable diagnostic delay. We conducted the current study to assess the incidence and clinicoradiologic characteristics of idiopathic RPF. For this, we evaluated prospectively 53 consecutive patients with a diagnosis of idiopathic RPF at our tertiary care referral center from April 1998 through January 2008.Calculated annual incidence of RPF was 1.3/100,000 inhabitants. Mean age was 64 +/- 11.1 (SD) yr; male-female ratio was 3.3:1.0. Median duration of symptoms was 6.0 mo (IQR 3.0-12.0). Abdominal, flank, and/or back pain and discomfort were the major symptoms, with visual analogue scale scores of 49 +/- 27.2 mm and 43 +/- 29.4 mm for pain and discomfort, respectively. Female patients had higher erythrocyte sedimentation rate (ESR), higher white blood cell count, and lower hemoglobin content than male patients at presentation. Computed tomography-documented maximal mass thickness amounted to 35 +/- 16.6 mm; craniocaudal length amounted to 137 +/- 48.8 mm. RPF mass extension up to or above the level of the renal vessels was noted in 3 patients (6%). Six patients (11%) presented with atypical RPF localization and/or bulky mass. Localized lymphadenopathy adjacent to the RPF mass was observed frequently (25%). Patients with hydronephrosis (56%) presented earlier than patients without hydronephrosis, with higher creatinine and greater mass thickness but similar pain severity. Patients were typically at high cardiovascular risk with increased-often aneurysmal-infrarenal aortic diameter (25.0 mm, IQR 22.0-30.0). RPF mass distribution was similar in patients with or without aneurysmal dilation. Occupational asbestos exposure (20%) and asbestos-related pleural changes (17%) were frequent among males. Previous or concurrent chronic inflammatory disease and/or autoimmune disease was noted in 8 patients (15%). Multivariate analysis revealed an independent association of ESR values with severity of pain and discomfort. Smoking was independently associated with infrarenal aortic diameter.In summary, annual RPF incidence is higher than previously assumed. Age at diagnosis and male-female ratio seem to have changed over time. RPF typically affects patients at high cardiovascular risk, including increased aortic diameter. Clinical presentation is influenced by sex, severity of inflammation and presence of hydronephrosis. Prolonged asbestos exposure and asbestos-related pleural changes were frequent among males. Localized lymphadenopathy adjacent to the RPF mass occurs frequently and should not confuse RPF diagnosis.

In plain English

This prospective study was carried out at a referral hospital in Dordrecht, the Netherlands. It followed 53 consecutive patients diagnosed with idiopathic retroperitoneal fibrosis (RPF), a rare disorder in which fibrous tissue forms in the back of the abdomen, from April 1998 through January 2008. Of the 53 patients, 41 were men. Counting only 37 new cases from the hospital's own area of 290,000 people, the authors calculated an incidence of 1.3 cases per 100,000 inhabitants per year. Pain was the most common symptom, reported by 92% of patients. Most patients had cardiovascular risk factors or established cardiovascular disease. Patients were asked about asbestos exposure, and eight of the 41 men (20%) had been exposed at work. CT scans showed pleural plaques or pleural thickening in 7 of the 41 men (17%), and six of these 7 had occupational asbestos exposure. No evidence of mesothelioma was found in these patients. The authors concluded that RPF incidence was higher than previously assumed, and that prolonged asbestos exposure and asbestos-related pleural changes were frequent among the male patients.

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: Prospective single-center study of consecutive patients (case series)
• Who was studied: 53 consecutive patients diagnosed with idiopathic RPF (41 men) at the Albert Schweitzer Hospital, Dordrecht, the Netherlands, from April 1998 through January 2008; 37 were new cases from the hospital's catchment area of 290,000 inhabitants
• Exposure: Self-reported occupational asbestos exposure, asked about at presentation; eight patients, all men (20% of the 41 male patients), reported it. No fiber types or doses were given. Pleural abnormalities were recorded on CT scans of the basal lung regions.
• Main results:
  • Annual incidence of RPF in the hospital's catchment area: 1.3 cases per 100,000 inhabitants (37 new cases over 9.7 years)
  • 41 of the 53 patients were male; male-female ratio 3.3 to 1.0
  • Lower back, abdominal and/or flank pain was the most common symptom, in 92% of patients
  • Cardiovascular risk factors and/or established cardiovascular disease were present in most patients
  • Eight patients, all male, had been occupationally exposed to asbestos, which is 20% of the 41 male patients
  • Pleural plaques or pleural thickening on CT in 7 of the 41 male patients (17%); 2 had extensive bilateral calcified pleural plaques
  • Six of the 7 men with pleural changes had been occupationally exposed to asbestos
  • No evidence of mesothelioma was found in the 7 men with pleural changes
  • Current smoking remained significantly associated with infrarenal aortic diameter after adjustment (regression coefficient 7.3; 95% CI 1.5 to 13.1; p = 0.02)
• The authors' conclusion: The authors concluded that annual RPF incidence is higher than previously assumed. They also concluded that prolonged asbestos exposure and asbestos-related pleural changes were frequent among male patients, which they described as further evidence for an association of RPF with occupational asbestos exposure.

Topics

Medical terms in this study, explained in our Lexicon: Idiopathic, Fibrosis, Etiology, Lymphadenopathy, Asbestos Exposure, Multivariate Analysis, Inflammation.

Related studies

• 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)
• Idiopathic retroperitoneal fibrosis: an update for nephrologists — V. Raglianti, G.M. Rossi, et al. (2021)
• Asbestos and idiopathic retroperitoneal fibrosis — J.C. Boulard, T. Hanslik, et al. (1995)
• Asbestos exposure: a potential cause of retroperitoneal fibrosis — R. Sauni, P. Oksa, et al. (1998)
• 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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