IGG4-Related Pleural Bridging Fibrosis, Retrosternal Fibrosclerosis and Retroperitoneal Fibrosis in an Asbestos-Exposed Patient

Our plain-English summary of IGG4-Related Pleural Bridging Fibrosis, Retrosternal Fibrosclerosis and Retroperitoneal Fibrosis in an Asbestos-Exposed Patient, by P.P.T.E.S. Torres, L.B.M. Fonseca, K.S. Teixeira, et al (Archivos de Bronconeumología, 2019).

The study

• Authors: P.P.T.E.S. Torres, L.B.M. Fonseca, K.S. Teixeira, M.F. Rabahi, E. Marchiori
• Journal: Archivos de Bronconeumología
• Published: 2019;55(9):496-497
• Type of article: Case report, Letter
• Language: Spanish
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Summary of the article

In this letter, the authors described a 78-year-old man with a history of 13 years of occupational asbestos exposure who presented with shortness of breath (dyspnea). He had packed asbestos-containing waste materials until the age of 50, giving a latency period of 28 years. Chest computed tomography (CT) showed an irregular, uncalcified pleural plaque in the front of the right side of the chest. One year later the plaque had flattened and extended to the area behind the breastbone (retrosternal mediastinum) and to the pleural space on the other side. A small soft-tissue mass around the abdominal aorta below the kidneys was also found. A biopsy taken through the chest wall showed storiform fibrosis with polyclonal lymphoplasmacytic and plasma cell infiltration. Plasma cells expressed immunoglobulin G4 (IgG4), with an IgG4/IgG ratio above 50%. The serial IgG4 concentration was high (5920 mg/L). The final diagnosis was IgG4-related bridging pleural fibrosis with retrosternal fibrosclerosis, together with a subtle retroperitoneal soft-tissue mass. The authors noted that typical asbestos-related pleural fibrosis appears as bilateral, partially calcified plaques, which is quite different from this patient's findings. They reviewed reports suggesting that asbestos might trigger IgG4-related disease through allergic-type immune responses. They concluded that the case highlighted, as previously suggested, a possible association between asbestos exposure and these conditions.

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 letter is a case report about one patient. The authors described a 78-year-old man with 13 years of past asbestos exposure who came to medical attention with shortness of breath (dyspnea). He had packed asbestos-containing waste materials until age 50, and the authors gave a latency period of 28 years. A chest CT scan showed an irregular, uncalcified pleural plaque at the front of the right side of the chest. One year later the plaque had flattened and extended behind the breastbone and into the pleural space on the other side. A small soft-tissue mass around the abdominal aorta below the kidneys was also seen. A needle biopsy through the chest wall showed storiform fibrosis with inflammatory cell infiltration. Plasma cells expressed IgG4, with an IgG4 to IgG ratio greater than 50%. The blood IgG4 level was high (5920 mg/L). The diagnosis was IgG4-related bridging pleural fibrosis with retrosternal fibrosclerosis. The authors noted that this pleural thickening differed from typical asbestos-related plaques. They concluded that the case highlights, as previously suggested, a possible association between these conditions and occupational asbestos exposure.

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 (letter to the editor)
• Who was studied: One 78-year-old man with dyspnea and a history of occupational asbestos exposure. The authors are based in Brazil. The article does not state where or when the patient was seen.
• Exposure: 13 years of occupational asbestos exposure from packing asbestos-containing waste materials until age 50, with a latency period of 28 years. The fiber type is not stated.
• Main results:
  • Chest CT showed an irregular, uncalcified pleural plaque in the anterior right hemithorax.
  • One year later the plaque had flattened and extended to the retrosternal mediastinum and the pleural space on the opposite side.
  • A small soft-tissue mass surrounding the abdominal infrarenal aorta was also identified.
  • Transthoracic percutaneous biopsy showed storiform fibrosis with polyclonal lymphoplasmocytic and plasma cell infiltration. Plasma cells expressed IgG4, with an IgG4 to IgG ratio greater than 50%.
  • The serum IgG4 concentration was high (5920 mg/L). The final diagnosis was IgG4-related bridging pleural fibrosis with retrosternal fibrosclerosis.
  • The authors noted that the pleural thickening in this patient differed from usual asbestos-related pleural fibrosis, and that the mediastinal fibrosis imaging findings were also atypical.
• The authors' conclusion: The authors concluded that they had described a clinicopathologically proven case of IgG4-related bridging pleural fibrosis, retrosternal fibrosclerosis and retroperitoneal fibrosis in an occupationally asbestos-exposed patient. In their view the case highlights, as previously suggested, a possible association between these conditions.

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, Dyspnea, Latency, Pleural Plaques, Mediastinum, Biopsy, Bilateral.

Related studies

• Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure — V. Cottin, P.Y. Brillet, et al. (2008)
• High serum immunoglobulin G4-related retrosternal fibrosclerosis — K. Kamiya, A. Yoshizu, et al. (2012)
• Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis — T. Uibu, R. Järvenpää, et al. (2008)
• Retrosternal and pleural "bridging" fibrosis mimicking malignancy in a patient exposed to asbestos — M.P. Debray, S. Marchand-Adam, et al. (2010)
• Asbestos and idiopathic retroperitoneal fibrosis — J.C. Boulard, T. Hanslik, et al. (1995)
• Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation — E.F.H. van Bommel, I. Jansen, et al. (2009)

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