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

Our plain-English summary of IgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review, by S. Okamoto, H. Tsuboi, R. Sato, et al (Rheumatology International, 2020).

The study

• Authors: S. Okamoto, H. Tsuboi, R. Sato, M. Terasaki, T. Terasaki, H. Toko, M. Shimizu, F. Honda, M. Yagishita, A. Ohyama, I. Kurata, S. Abe, H. Takahashi, A. Osada, S. Hagiwara, Y. Kondo, I. Matsumoto, T. Sumida
• Journal: Rheumatology International
• Published: 2020;40(10):1725-1732
• Type of article: Case report, Review
• Cited by: 7 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

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.

In plain English

This article was a case report combined with a review of published cases. A 64-year-old Japanese man had back pain, and scans showed thickening of the right pleura (the lining around the lung) and tissue around the abdominal aorta. Malignant pleural mesothelioma was suspected from the imaging, and the article noted that any history of asbestos exposure was unclear. A pleural biopsy excluded malignancies such as malignant pleural mesothelioma, and also infections. It showed fibrosis and IgG4-positive plasma cells. His blood IgG4 was raised (570 mg/dl; reference 4.5 to 117). He was diagnosed with IgG4-related disease involving the pleura, the aorta and both submandibular glands. After treatment with the steroid prednisolone, scans at 6 months showed dramatic shrinkage of the pleural and aortic lesions, with no evidence of recurrence for 10 months. The authors also reviewed 16 earlier cases confirmed by pleural biopsy. Across all 17 cases, pleural fluid was the most common finding (15 of 17). The authors concluded that IgG4-related pleural disease could be considered when pleural lesions have an unknown cause, and that pleural biopsy must be done in every pleural involvement.

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 with a literature review of previously reported cases
• Who was studied: One 64-year-old Japanese man treated at the University of Tsukuba, Japan, plus 16 previously reported cases of IgG4-related pleural disease confirmed by pleural biopsy, found in PubMed/MEDLINE articles published between January 2008 and February 2019
• Exposure: No asbestos exposure was established; the patient's history of asbestos exposure was unclear. The condition studied was IgG4-related disease, not an asbestos-related disease.
• Main results:
  • In the patient, the pleural biopsy excluded malignancies such as malignant pleural mesothelioma, and also infections. It showed marked fibrosis and IgG4-positive plasma cell infiltration, with a 40% IgG4-positive to IgG-positive plasma cell ratio.
  • The patient's serum IgG4 was elevated at 570 mg/dl at first testing (reference value 4.5 to 117 mg/dl) and was 538 mg/dl on admission.
  • The patient's pleural specimen showed 20 to 30 IgG4-positive plasma cells per high-power field, with no storiform fibrosis and no obliterative phlebitis.
  • The patient was started on oral prednisolone 30 mg/day (0.6 mg/kg/day). CT 6 months later showed dramatic regression of the pleural thickening and the periaortic soft tissue. There was no evidence of recurrence for 10 months at the last visit.
  • In the literature search, 33 articles were found, and 16 of them (describing 16 cases) met the inclusion criteria.
  • Across the 17 cases (16 published cases plus this one), 12 of 17 (70.6%) were male. The mean age was 63.7 plus or minus 13.3 years.
  • Across the 17 cases, pleural effusion was the most common pleural finding (15 of 17, 88.2%), followed by pleural thickening (8 of 17, 47.1%).
  • Across the 17 cases, storiform fibrosis was seen in 4 of 17 (23.5%) and obliterative phlebitis in 1 of 17 (5.9%).
  • Periaortitis and submandibular gland swelling were found only in the present case. Four of the 17 patients had only pleural lesions.
• The authors' conclusion: The authors concluded that IgG4-related pleural disease could be a differential diagnosis in patients with pleural lesions of unknown cause, and that pleural histopathological examination must be done in every pleural involvement. They also concluded that the clinical findings improve with initial corticosteroid treatment.

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Pleural Mesothelioma, Bilateral, Pleural Thickening, Pleura.

Related studies

• Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma — F. Lococo, T. Di Stefano, et al. (2019)
• IgG4-Related Lung Disease Exhibiting the Invasion into the Diaphragm: A Case Report — Y. Ono, G. Toyokawa, et al. (2022)
• 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)
• Chronic periaortitis: a spectrum of diseases — A. Vaglio, C. Buzio (2005)
• Ventilatory insufficiency due to asbestos-related diffuse pleural fibrosis successfully treated with noninvasive home mechanical ventilation — X. Muñoz, A. Roger, et al. (2001)
• Giant solitary fibrous tumor of the liver with metastasis to the skeletal system successfully treated with trisegmentectomy — S. Yilmaz, V. Kirimlioglu, et al. (2000)

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