Medical Challenge Posed by Retroperitoneal Fibrosis: Case Reports and Literature Review

Our plain-English summary of Medical Challenge Posed by Retroperitoneal Fibrosis: Case Reports and Literature Review, by S. Cervera-Bonilla, M. Garcia Mora, P. Rodriguez Ossa, et al (Curēus, 2020).

The study

• Authors: S. Cervera-Bonilla, M. Garcia Mora, P. Rodriguez Ossa, O. Messa, S. Mendoza Díaz
• Journal: Curēus
• Published: 2020;12(1):e6624
• Type of article: Case report
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Idiopathic retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease, with a low incidence worldwide, which occurs around the abdominal aorta and the iliac arteries. It spreads through the retroperitoneum causing ureteral obstruction with associated renal failure and obstruction of other adjacent structures. RPF can be idiopathic or secondary to neoplastic processes, infections, or medications. RPF is considered part of the spectrum of the disease related to immunoglobulin G4 (IgG4) and other autoimmune disorders. Occupational exposure to asbestos and tobacco smoke are important risk factors for the development of idiopathic RPF. The clinical picture is nonspecific, from pain to symptoms due to ureteral compression, this being the main complication associated. Imaging studies are essential in the diagnosis; computed tomography (CT) and magnetic resonance imaging (MRI) are the most reliable imaging modalities. The goal of treatment is to stop the progression of the fibroinflammatory reaction. The first line of treatment is usually with medical management. Biological agents, such as rituximab and infliximab, have also been used, even with scarce data in the literature. Surgery is usually performed to improve a ureteral obstruction and should always be accompanied by systemic steroid treatment. The conservative approach given by systemic therapy and ureteral stent placement or nephrostomies has been recommended, thus reserving surgical treatment for refractory cases. We present two clinical cases of idiopathic RPF, one of them associated with IgG4.

Copyright © 2020, Cervera-Bonilla et al.

In plain English

This article reported two patients with retroperitoneal fibrosis (RPF), a rare condition in which fibrous, inflamed tissue forms around the abdominal aorta, and reviewed the literature. The first patient, a 54-year-old man with left flank pain, had a mass larger than 4 cm around the abdominal aorta that could not be removed. After a biopsy, he was diagnosed with a lesion compatible with IgG4-related disease and did well after surgery. The second patient, a 48-year-old man with left abdominal pain, had a 99 x 38 x 55 mm mass and left hydronephrosis, a swelling of the kidney from poor urine drainage. His blood IgG4 test was negative. He was diagnosed with idiopathic RPF and given corticosteroids, and his disease did not progress during follow-up. The authors reported no environmental, infectious or medication-related associations in their cases. Asbestos appeared only as background from the literature, not as an exposure in these patients: the authors cited another study naming occupational asbestos and tobacco smoke as risk factors for idiopathic RPF. The authors concluded that IgG4-related disease should always be ruled out.

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: Case reports (two patients) with a literature review
• Who was studied: Two men, aged 54 and 48, with retroperitoneal masses. The authors are affiliated with the Instituto Nacional de Cancerología, Bogotá, Colombia. Dates of care are not given.
• Exposure: The authors did not report asbestos exposure for either patient. They stated that their cases had no environmental, infectious or medication-related associations. Asbestos appears only as background drawn from the literature, in the abstract and in the review section, where it is attributed to another study (reference 9).
• Main results:
  • Case 1 (54-year-old man): an unresectable mass larger than 4 cm surrounded the infrarenal abdominal aorta. After biopsy, it was diagnosed as a lesion compatible with IgG4-related disease.
  • Case 1: the tissue showed areas with more than 50 IgG4 plasma cells per field, but the ratio of IgG4 to IgG cells was not greater than 40%.
  • Case 1 had a good clinical course after surgery and was followed as an outpatient.
  • Case 2 (48-year-old man): a retroperitoneal mass measuring 99 x 38 x 55 mm with left hydronephrosis.
  • Case 2: the serum IgG4 test was negative, and the diagnosis was idiopathic RPF.
  • Case 2 was treated with corticosteroids (dose printed as 1 mg/day) and showed no progression during follow-up.
  • Literature review only, not a finding of this study: the authors cited reference 9 (Uibu et al., Lancet 2004) for occupational asbestos exposure and tobacco smoke as risk factors for idiopathic RPF.
• The authors' conclusion: The authors concluded that RPF is rare and difficult to diagnose, and that IgG4-related disease should always be ruled out.
• Limitations the authors state:
  • The authors noted that the pathology findings in case 1 were not specific for IgG4-related disease and that the IgG to IgG4 ratio was not as expected.

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Idiopathic, Retroperitoneum, Occupational Exposure, Systemic Therapy.

Related studies

• Retroperitoneal fibrosis associated with orbital pseudotumor without evidence of IgG4: A case report with review of literature — M.T. Pérez-Sanz, E. Cervilla-Muñoz, et al. (2019)
• Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation — E.F.H. van Bommel, I. Jansen, et al. (2009)
• Asbestos exposure as a risk factor for retroperitoneal fibrosis — T. Uibu, P. Oksa, et al. (2004)
• 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)
• Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis — A. Vaglio (2009)

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