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Clinical significance of immunoglobulin G4-associated autoimmune hepatitis

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Abstract

Background

Immunoglobulin (Ig) G4-associated autoimmune hepatitis (AIH) is a recently identified and possibly new disease entity. However, the epidemiology and clinical features of IgG4-associated AIH remain uncertain. The aim of this study was to determine the prevalence and the clinical, serological, and histological characteristics of IgG4-associated AIH.

Methods

We examined the clinical features, serum IgG4 concentration, liver biopsy histology, and IgG4-bearing plasma cell infiltration of 60 patients with type 1 AIH and 22 patients with autoimmune pancreatitis.

Results

High serum IgG4 concentration (≥135 mg/dL) and IgG4-bearing plasma cell infiltration in the liver (≥10/high-power fields [HPFs]) were found in 2 of the 60 (3.3%) patients with type 1 AIH. These patients had high serum levels of IgE, giant cell change, and rosette formation in the liver. Although corticosteroid therapy reduced the serum IgG4 concentration and normalized liver enzymes and histology, one patient developed IgG4-related sclerosing cholangitis after 5 years of follow-up.

Conclusions

Because IgG4-associated AIH was found in over 3% of Japanese patients with type 1 AIH in our cohort, further studies are needed on this possible new disease entity and its impact on the diagnostic guidelines of AIH.

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Acknowledgments

The authors would like to thank Mr. Trevor Ralph for his editorial assistance, and Professor Ian R. Mackay for critically reading this manuscript. This study was funded in part by a research grant from the Japanese Ministry of Health, Labour, and Welfare and a Shinshu University Grant-in-Aid for Young Scientists in Exploratory Research.

Author information

Author notes

  1. Yoh Zen

    Present address: Institute of Liver Studies, King’s College Hospital, London, UK

  2. Kendo Kiyosawa

    Present address: Department of Internal Medicine, Nagano Red Cross Hospital, Nagano, Japan

Authors and Affiliations

  1. Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Takeji Umemura, Hideaki Hamano, Satoru Joshita, Tetsuya Ichijo, Kaname Yoshizawa, Kendo Kiyosawa & Eiji Tanaka

  2. Department of Legal Medicine, Shinshu University School of Medicine, Matsumoto, Japan

    Masao Ota

  3. Center for Health, Safety, and Environmental Management, Shinshu University, Matsumoto, Japan

    Shigeyuki Kawa

  4. Division of Pathology, Kanazawa University Hospital, Kanazawa, Japan

    Yoh Zen

  5. Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Yasuni Nakanuma

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  1. Takeji Umemura
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Correspondence to Takeji Umemura.

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Umemura, T., Zen, Y., Hamano, H. et al. Clinical significance of immunoglobulin G4-associated autoimmune hepatitis. J Gastroenterol 46 (Suppl 1), 48–55 (2011). https://doi.org/10.1007/s00535-010-0323-4

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  • DOI: https://doi.org/10.1007/s00535-010-0323-4

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