IgG4-Related Lung Disease Exhibiting the Invasion into the Diaphragm: A Case Report

Our plain-English summary of IgG4-Related Lung Disease Exhibiting the Invasion into the Diaphragm: A Case Report, by Y. Ono, G. Toyokawa, T. Tagawa, et al (Annals of Thoracic and Cardiovascular Surgery, 2022).

The study

• Authors: Y. Ono, G. Toyokawa, T. Tagawa, K. Ijichi, Y. Oda, M. Mori
• Journal: Annals of Thoracic and Cardiovascular Surgery
• Published: 2022;28(3):223-226
• Type of article: Case report
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition which involves various organs. This is a very rare case of IgG4-related lung disease (IgG4-RLD) with the invasion into diaphragm. The patient was a 71-year-old man with a long-term exposure to asbestos who had a mass shadow in the left lower lung lobe, which was suspected to invade the left diaphragm on computed tomography (CT). Positron emission tomography (PET)/CT also presented an avid intake of fluorodeoxyglucose in the mass, which suspected lung cancer. Although bronchoscopic biopsy could not lead to the definite diagnosis, we performed left lower lobectomy combined with the resection of left diaphragm. The specimen showed the features of IgG4-RLD on pathology: the vein stenosis and fibrosis around the vein, the infiltration of IgG4-positive cells, and IgG cells to IgG4 cells ratio of 40%. Furthermore, there were inflammatory cells infiltrating to the diaphragm.

In plain English

This was a case report of one patient: a 71-year-old man who had done cement work, had a smoking history, and regularly saw a doctor for what the article calls "asbestos lung." No asbestos fiber type was named. A CT scan showed a 63 mm mass in the lower lobe of the left lung that seemed to reach the diaphragm, and a PET scan showed strong uptake in the mass. A biopsy through the airway found no cancer. Lung cancer was still suspected before surgery, and a sample taken during the operation was read as adenocarcinoma. Surgeons removed the left lower lobe and part of the diaphragm. Examination of the removed tissue found no cancer. Instead it showed features of IgG4-related lung disease, an inflammatory and scarring condition, with inflammatory cells reaching into the diaphragm. Because the patient had no atopic (allergic) disease, the authors wrote that asbestos exposure "may be the trigger." They concluded that this disease can be difficult to tell apart from lung cancer and can mislead doctors.

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), with a brief discussion of earlier published reports
• Who was studied: one 71-year-old man who had done cement work and had a smoking history the article prints as "20 packs per a year"; seen at Kyushu University surgery and pathology departments, Japan
• Exposure: cement work; the patient was regularly followed by a doctor for "asbestos lung" and is described as having long-term asbestos exposure; no fiber type or dose given
• Main results:
  • CT showed a 63 mm mass in the left lower lung lobe that possibly invaded the adjacent diaphragm
  • PET scan showed high FDG uptake, with maximum standardized uptake value 25.6 in the mass and 4.83 in the hilar lymph nodes
  • Transbronchial biopsy found no malignant tissue, but the patient was diagnosed before surgery with suspected lung cancer (T3N1M0, Stage IIIB)
  • Aspiration cytology during the operation was read as class V adenocarcinoma, and left lower lobectomy with resection and reconstruction of the left diaphragm was performed
  • The resected tissue showed no malignancy, with vein stenosis, fibrosis around the vein, plasma cell infiltration and a high IgG4/IgG ratio (figure legend: immunoglobulin G to immunoglobulin G4 ratio of 40%)
  • Inflammatory cells had invaded the diaphragm
  • With serum IgG4 remaining higher even after surgery, the final diagnosis was IgG4-related lung disease
  • The patient was discharged on day 11 after surgery without complications
  • The authors suggested that, because the patient had no atopic disease, asbestos exposure may have triggered the IgG4-related lung disease
• The authors' conclusion: The authors concluded that this was an extremely rare case of IgG4-related lung disease invading the diaphragm, that the disease can be hard to distinguish from lung cancer and may mislead, and that further research and more cases are needed

Topics

Medical terms in this study, explained in our Lexicon: Diaphragm, Lung Cancer, Biopsy, Pathology, Fibrosis.

Related studies

• Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma — F. Lococo, T. Di Stefano, et al. (2019)
• IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review — S. Okamoto, H. Tsuboi, et al. (2020)
• Efficacy of intra-operative unilateral diaphragm plication for patients undergoing unilateral phrenicotomy during extended surgery — T. Tokunaga, N. Sawabata, et al. (2010)
• Primary malignant mesothelioma of the diaphragm with liver invasion: A case report and review of literature — W.J. Huang, Z.H. Li, et al. (2019)
• 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)
• Effect of asbestos-related pleural fibrosis on excursion of the lower chest wall and diaphragm — B. Singh, P.R. Eastwood, et al. (1999)

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