Retroperitoneal fibrosis associated with orbital pseudotumor without evidence of IgG4: A case report with review of literature

Our plain-English summary of Retroperitoneal fibrosis associated with orbital pseudotumor without evidence of IgG4: A case report with review of literature, by M.T. Pérez-Sanz, E. Cervilla-Muñoz, J. Alonso-Muñoz, et al (Intractable & Rare Diseases Research, 2019).

The study

• Authors: M.T. Pérez-Sanz, E. Cervilla-Muñoz, J. Alonso-Muñoz, A. Marcelo-Ayala, M.D. Pulfer, F. Galeano-Valle
• Journal: Intractable & Rare Diseases Research
• Published: 2019;8(1):29-35
• Type of article: Case report
• Cited by: 3 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic inflammation and periaortic fibrosis that affects retroperitoneal structures and often entraps the ureters. The idiopathic form has an incidence of 0.1-1.3/100,000 person-years. A substantial percentage of patients with idiopathic retroperitoneal fibrosis (IRF), as well as patients with orbital pseudotumor, is associated with IgG4-related disease (IgG4-RD). It is not clear what percentage of IRF is related to the spectrum of the IgG4-RD or if both represent different stages of the same disease (especially in those cases with extra-retroperitoneal involvement). Histopathological features such as storiform fibrosis, obliterative phlebitis and tissue infiltration of IgG4-positive plasma cells (ratio IgG4+/IgG higher than 0.4) are essential to identify this association. Extra-retroperitoneal manifestations are often presented among patients with IgG4-related RPF. About 90% of cases of IRF have a good prognosis, with adequate response to treatment. We report a case of a 59-year-old woman with history of past occupational asbestos exposure and smoking habit. She was diagnosed with RPF, periaortitis and orbital pseudotumor, without histopathologic or serologic features of IgG4- related disease. This could be related to the fact that the biopsy was done in a place with scarce inflammatory activity but high fibrosis. We want to emphasize the usual need to perform several biopsies or to be guided by positron emission tomography (PET-CT) in order to achieve a histopathological confirmation. Our case differs from the main IgG4 international cohorts in the involvement of the retroperitoneum, aorta and eye, whereas the usual involvement includes liver, pancreas, lymph nodes and salivary glands. Our patient had lower IgG4 serum levels than those described in the international cohorts. However, they were similar to those of the Spanish population.

In plain English

This article is a case report with a short review of published case series. It described a 59-year-old woman with a history of occupational asbestos exposure and smoking. The article did not describe her job, the asbestos fiber type or how long she was exposed, and it did not examine whether asbestos caused her illness. Scans showed a soft tissue mass around the aorta that spread into the retroperitoneum (the area at the back of the abdomen) and blocked both ureters. They also showed inflammation of the tissue around the eyes, suggestive of orbital pseudotumor. A biopsy of the mass showed fibrosis, and staining did not reveal IgG4. Her blood IgG4 level was within normal range. The findings were compatible with idiopathic retroperitoneal fibrosis. She improved with high-dose steroids. She was later admitted to intensive care with abdominal sepsis from a urinary tract infection and colitis, developed multiple organ failure, and died. The authors suggested the biopsy may have missed IgG4 features because it came from heavily scarred tissue. They stressed the need for several biopsies or PET-CT guidance.

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 report with a review of published IgG4-related disease case series
• Who was studied: one 59-year-old woman with a history of occupational asbestos exposure and smoking; the article gives no place or dates of care
• Exposure: past occupational asbestos exposure (no fiber type, job, duration or dose given) and smoking
• Main results:
  • CT in the patient showed a soft tissue mass around the aortic arch, descending aorta and iliac arteries that infiltrated the retroperitoneum and caused bilateral obstructive uropathy
  • Imaging also showed inflammation of the orbital soft tissues in the patient, suggestive of orbital inflammatory pseudotumor
  • The patient's serum IgG4 was 0.18 g/dL, within normal range
  • Biopsy staining of the retroperitoneal mass did not reveal IgG4, and the findings were compatible with idiopathic retroperitoneal fibrosis
  • After intravenous methylprednisolone (1 g/day for 3 days), the patient's symptoms improved, including resolution of proptosis, fever, vomiting, ascites and abdominal pain
  • After discharge, the patient was admitted to intensive care with abdominal sepsis from a urinary tract infection and pseudomembranous colitis
  • The patient developed multiple organ failure and died
  • In the literature review, the authors found ten IgG4-related disease case series and included six in their comparison table
  • Compared with those cohorts, this patient's disease involved the retroperitoneum, aorta and eye, rather than the organs most often affected in the cohorts
  • The patient's serum IgG4 was lower than in the international cohorts but similar to the Spanish cohort
• The authors' conclusion: The authors suggested the absence of IgG4 features may have been because the biopsy came from an area with little inflammation and much fibrosis, and emphasized the need for several biopsies or PET-CT guidance to confirm the diagnosis
• Limitations the authors state:
  • Only one biopsy was performed, and the disease can affect tissue unevenly, so IgG4 features may have been missed

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Chronic Inflammation, Idiopathic, Prognosis, Asbestos Exposure, Habit, Biopsy, PET-CT.

Related studies

• Medical Challenge Posed by Retroperitoneal Fibrosis: Case Reports and Literature Review — S. Cervera-Bonilla, M. Garcia Mora, et al. (2020)
• 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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