High serum immunoglobulin G4-related retrosternal fibrosclerosis

Our plain-English summary of High serum immunoglobulin G4-related retrosternal fibrosclerosis, by K. Kamiya, A. Yoshizu, T. Nakazato, et al (Journal of Thoracic Imaging, 2012).

The study

• Authors: K. Kamiya, A. Yoshizu, T. Nakazato, H. Hayashi, N. Hida, H. Okamoto
• Journal: Journal of Thoracic Imaging
• Published: 2012;27(6):W190-2
• Type of article: Case report
• Cited by: 7 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

A 65-year-old man with a history of exposure to asbestos complained of left leg edema. Computed tomography showed a flat, symmetrical, and longitudinal retrosternal thickening in addition to a presacral tumor. Retroperitoneal fibrosis was suspected. Serum immunoglobulin G4 (IgG4) levels were elevated (213 mg/dL). Thoracoscopic biopsy was performed. Histopathologic findings showed fibrotic tissue accompanied by proliferation of IgG4-positive plasma cells (proportion of IgG4/IgG-positive plasma cells ≥70%) indicative of multifocal fibrosclerosis.

In plain English

This case report described one 65-year-old man in Japan with left leg swelling. He had smoked and had a job history that included 5 years of asbestos exposure. The article did not name a fiber type. Blood tests showed raised IgG and IgG4 antibody levels. CT scans showed a flat thickening about 20 cm long behind the breastbone and a flat tumor in front of the sacrum (base of the spine). CT showed no abnormalities elsewhere, including the pleura. A biopsy found scar-like fibrous tissue with many IgG4-positive plasma cells. He was diagnosed with IgG4-related multifocal fibrosclerosis, scarring in several sites. Untreated, the disease progressed; three months later he developed renal (kidney) dysfunction. After ureteral stents and the steroid prednisolone, kidney function improved, the tumor and the thickening both shrank, and the leg swelling resolved. The authors' Medline search through PubMed found no reports of this flat, symmetrical, lengthwise thickening behind the breastbone. The authors concluded that asbestos exposure may be related to these conditions and that future studies are needed. A single case cannot show a cause or a rate.

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 (one patient)
• Who was studied: One 65-year-old man with left leg edema, seen at Yokohama Municipal Citizen's Hospital, Japan
• Exposure: Occupational asbestos exposure for 5 years (fiber type not stated). He also had a smoking history of 30 cigarettes per day for 15 years.
• Main results:
  • In this patient, serum IgG was 2096 mg/dL (reference 800 to 1600 mg/dL) and serum IgG4 was 213 mg/dL (reference below 80 mg/dL)
  • CT showed a flat, longitudinal thickening measuring 20 cm behind the sternum, plus a presacral flat tumor
  • CT showed no abnormalities in other regions, including the pleura and pancreas
  • Thoracoscopic biopsy showed fibrotic tissue with increased IgG4-positive plasma cells (70% or more of IgG-positive plasma cells), and the patient was diagnosed with IgG4-related multifocal fibrosclerosis
  • Untreated, the disease progressed. Three months later the patient developed renal dysfunction (blood urea nitrogen 97.1 mg/dL; creatinine 12.98 mg/dL) with bilateral hydronephrosis.
  • After bilateral ureteral stents and prednisolone 60 mg/day (tapered), renal function improved gradually. At 6 months CT showed a decrease in the size of the presacral tumor and the retrosternal thickening, and the left leg edema had resolved.
  • The authors' Medline/PubMed search found no prior reports of flat, symmetrical, longitudinal retrosternal thickening in the anterior mediastinum
• The authors' conclusion: The authors concluded that a history of asbestos exposure may be related to multifocal fibrosclerosis or IgG4-related disease, and that future studies are needed to investigate this possibility

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Thoracoscopic Biopsy.

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)
• Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure — V. Cottin, P.Y. Brillet, et al. (2008)
• An epidemiological survey of immunological abnormalities in asbestos workers. II. Serum immunoglobulin levels — A. Lange (1980)
• Binding of immunoglobulin and activation of complement by asbestos fibers — P. Hasselbacher (1979)
• Enhancement of the macrophage oxidative burst by immunoglobulin coating of respirable fibers: fiber-specific differences between asbestos and man-made fibers — I.M. Hill, P.H. Beswick, et al. (1996)
• Retrosternal and pleural "bridging" fibrosis mimicking malignancy in a patient exposed to asbestos — M.P. Debray, S. Marchand-Adam, et al. (2010)

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