Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure

Our plain-English summary of Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure, by V. Cottin, P.Y. Brillet, F. Combarnous, et al (Thorax, 2008).

The study

• Authors: V. Cottin, P.Y. Brillet, F. Combarnous, F. Duperron, H. Nunes, J.F. Cordier
• Journal: Thorax
• Published: 2008;63(2):177-9
• Type of article: Case report
• Cited by: 10 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Two case histories are described of pleural and anterior mediastinal fibrosis presenting as a continuous fibrotic process with thick parietal pleural plaques extending from one pleura to the contralateral pleura through the retrosternal area, and with retroperitoneal fibrosis. Follow-up over 4 years in one case demonstrated rapid progression of disease, with pleural fibrosis preceding retrosternal and retroperitoneal fibrosis. Histopathological analysis in both cases showed non-tumoral fibrosis with broad fibrous bundles surrounding fibroblasts (and lymphocytes in one case). Possible causes such as infections and exposure to ergot derivatives were excluded. Both patients had been slightly or moderately exposed to asbestos. These cases represent an unusual new presentation of pleural and retrosternal fibrosis extending beyond the anatomical structures and associated with retroperitoneal fibrosis.

In plain English

This article is a case report of two men in France. Each had fibrosis (scar-like tissue) of the pleura, the lining around the lungs, that ran continuously behind the breastbone from one side of the chest to the other. The authors called this "bridging" fibrosis. Both men also had retroperitoneal fibrosis, which is scar tissue at the back of the abdomen. The first man, aged 65, had been exposed to asbestos without respiratory protection for 5 years, thirty years earlier, and was admitted with acute kidney failure. The second man, aged 55, was a print worker exposed to lead and trichlorethylene who had also worked for 4 years in a building with asbestos-flocked walls. His chest scan in 2001 showed calcified pleural plaques. By 2005 a calcified mass behind the breastbone and signs of retroperitoneal fibrosis had appeared. Tissue samples from both men showed severe fibrosis. The authors described the asbestos exposure as mild to moderate and short term and said other potential causes were ruled out. They concluded that asbestos exposure may be considered a cause of this unusual presentation.

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 (two patients)
• Who was studied: Two men, aged 65 and 55, seen at hospitals in France; the second patient was followed from 2001 to October 2006
• Exposure: Asbestos exposure described by the authors as mild to moderate and short term. Case 1 had 5 years of exposure without respiratory protection. Case 2 worked 4 years in a building with asbestos-flocked walls and was also exposed to lead and trichlorethylene as a print worker. Fiber type is not stated.
• Main results:
  • Case 1 had been exposed to asbestos without respiratory protection for 5 years, thirty years before admission.
  • Case 2 had been exposed to lead and trichlorethylene as a print worker and had worked for 4 years in a building with asbestos-flocked walls.
  • In case 1, surgery found a hard fibrous mass at the back of the abdomen (retroperitoneum) adhering to nearby vessels.
  • In case 1, the chest CT showed calcified parietal pleural plaques and a thick calcified mass extending from one pleura to the other, involving the area behind the breastbone.
  • In case 1, biopsy of the retroperitoneal mass showed severe fibrosis, and a biopsy of the mass behind the breastbone looked very similar.
  • In case 1, 6 months of prednisone produced no change in the chest CT or lung function. After 4 months of tamoxifen, FVC and TLC (lung volume measures) each rose by 0.3 litres, and the chest CT was unchanged.
  • In case 2, the 2001 chest CT showed bilateral calcified circumferential pleural plaques. A pleural biopsy showed severe fibrosis with no granuloma, vasculitis or tumour.
  • In case 2, prednisone for 18 months improved symptoms and cleared the pleural effusion (fluid around the lung).
  • In case 2, by 2005 the CT showed a thick calcified mass behind the breastbone bridging from one pleura to the other. An abdominal CT suggested retroperitoneal fibrosis.
  • The authors stated that all other potential causes were ruled out, including raised IgG4. Serum IgG4 was normal in both patients.
• The authors' conclusion: The authors concluded that this syndrome of pleural and retrosternal 'bridging' fibrosis with retroperitoneal fibrosis was probably related to asbestos exposure, and that asbestos may be considered a causative agent in this unusual presentation.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Pleural plaques and thickening.

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

Related studies

• IGG4-Related Pleural Bridging Fibrosis, Retrosternal Fibrosclerosis and Retroperitoneal Fibrosis in an Asbestos-Exposed Patient — P.P.T.E.S. Torres, L.B.M. Fonseca, et al. (2019)
• Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis — T. Uibu, R. Järvenpää, et al. (2008)
• Asbestos and idiopathic retroperitoneal fibrosis — J.C. Boulard, T. Hanslik, et al. (1995)
• Retrosternal and pleural "bridging" fibrosis mimicking malignancy in a patient exposed to asbestos — M.P. Debray, S. Marchand-Adam, et al. (2010)
• Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation — E.F.H. van Bommel, I. Jansen, et al. (2009)
• Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis — A. Vaglio (2009)

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