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The Histopathology of IgG4-Related Disease

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IgG4-Related Disease

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 401))

Abstract

IgG4-related disease is a multi-organ immune-mediated chronic fibroinflammatory condition characterized by elevated serum IgG4 concentrations, tumefaction, and tissue infiltration by IgG4-positive plasma cells. The exact etiology of IgG4-related disease remains unclear with no known role of the IgG4 molecule itself being identified. Although the pancreas and salivary glands are the main organs affected, the involvement of other organs has also been reported. This multi-organ disease mimics a large number of malignant, infectious, and inflammatory disorders; therefore, a prompt differential diagnosis is important for selecting the right therapeutic strategy. Early steroid therapy assists in preventing tissue fibrosis, parenchymal extinction, and severe functional impairments in the affected organs. The definitive and prompt diagnosis of IgG4-related disease requires both histopathological confirmation and clinicopathological correlations. A histopathological examination is mandatory to exclude neoplastic or inflammatory conditions that mimic IgG4-related disease. The histological changes that occur are basically similar in any organ manifestation, with several site-specific findings being recognized. This chapter summarizes general rules for the pathological examination of IgG4-related disease, as well as the histopathological features and differential diagnoses of major organ manifestations.

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Authors and Affiliations

  1. Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-Cho, Kobe, 650-0017, Japan

    Mehmet Ozgur Avincsal & Yoh Zen

  2. Department of Otolaryngology, Kobe University Graduate School of Medicine, Kobe, Japan

    Mehmet Ozgur Avincsal

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  1. Mehmet Ozgur Avincsal
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  2. Yoh Zen
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Correspondence to Yoh Zen .

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Editors and Affiliations

  1. Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Osaka, Japan

    Kazuichi Okazaki

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Avincsal, M.O., Zen, Y. (2016). The Histopathology of IgG4-Related Disease. In: Okazaki, K. (eds) IgG4-Related Disease. Current Topics in Microbiology and Immunology, vol 401. Springer, Cham. https://doi.org/10.1007/82_2016_38

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