IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review

Rheumatology International volume 40, pages1725–1732(2020)Cite this article

Abstract

IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by increased serum IgG4 level, infiltration of lymphocytes and IgG4-positive (IgG4+) plasma cells and fibrosis. It can occur in almost all organs, commonly affecting the pancreas, biliary tract, salivary and lacrimal glands and kidneys. However, reports of IgG4-RD accompanied by pathologically confirmed, IgG4-related pleural disease are scarce. Here, we present a case of a 64-year-old man with suspected malignant pleural mesothelioma based on imaging findings but finally diagnosed with IgG4-RD (including pleuritis, periaortitis and bilateral submandibular gland enlargement) based on a high serum IgG4 level and pleural histopathological findings such as lymphoplasmacytic infiltration including IgG4+ plasma cells and fibrosis. Systemic corticosteroid therapy was effective at reducing serum IgG4, improving bilateral submandibular gland enlargement, and regressing pleural thickening and periaortic soft tissue. We also discuss clinical characteristics and pleural pathological features of previously reported cases with IgG4-related pleural disease based on a comprehensive literature review. Our case of IgG4-RD with pleura, aorta and submandibular gland involvement, pathologically confirmed by pleural specimen might be unique and very rare.

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Acknowledgements

We thank Dr. Bryan J. Mathis Medical English Communications Center, University of Tsukuba, for language review of this manuscript.

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Affiliations

  1. Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan

    Shota Okamoto, Hiroto Tsuboi, Ryota Sato, Mayu Terasaki, Toshihiko Terasaki, Hirofumi Toko, Masaru Shimizu, Fumika Honda, Mizuki Yagishita, Ayako Ohyama, Izumi Kurata, Saori Abe, Hiroyuki Takahashi, Atsumu Osada, Shinya Hagiwara, Yuya Kondo, Isao Matsumoto & Takayuki Sumida

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  1. Shota Okamoto
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Contributions

SO provided patient’s management, performed the literature review, and wrote the manuscript including editing and revision. HT and TS were involved in the clinical management and supervised the entire process from drafting the paper to submission of the final manuscript. All other authors have contributed to data collection and interpretation and critically reviewed the manuscript. All authors approved the final version of manuscript, and agree to accountable for all aspects of the work.

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Correspondence to Takayuki Sumida.

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The patient presented in this report gave his written informed consent prior to his inclusion.

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Okamoto, S., Tsuboi, H., Sato, R. et al. IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review. Rheumatol Int 40, 1725–1732 (2020). https://doi.org/10.1007/s00296-020-04555-y

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Keywords

  • IgG4-related disease
  • Pleural disease
  • Periaortitis
  • Submandibular gland