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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We thank Dr. Bryan J. Mathis Medical English Communications Center, University of Tsukuba, for language review of this manuscript.
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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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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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DOI: https://doi.org/10.1007/s00296-020-04555-y