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World J Gastroenterol. May 21, 2019; 25(19): 2308-2314
Published online May 21, 2019. doi: 10.3748/wjg.v25.i19.2308
Autoimmune hepatitis and IgG4-related disease
Kosuke Minaga, Tomohiro Watanabe, Hobyung Chung, Masatoshi Kudo
Kosuke Minaga, Tomohiro Watanabe, Masatoshi Kudo, Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka 589-8511, Japan
Hobyung Chung, Department of Gastroenterology and Hepatology, Kobe City General Hospital, Chuo-ku, Kobe 650-0047, Japan
Author contributions: Minaga K and Watanabe T wrote the manuscript draft and prepared the table. Chung H and Kudo M assisted in writing the manuscript and reviewed the final version.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Tomohiro Watanabe, MD, PhD, Associate Professor, Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan. tomohiro@med.kindai.ac.jp
Telephone: +81-72-3660221 Fax: +81-72-3672880
Received: March 8, 2019
Peer-review started: March 8, 2019
First decision: April 11, 2019
Revised: April 13, 2019
Accepted: April 19, 2019
Article in press: April 20, 2019
Published online: May 21, 2019
Abstract

IgG4-related disease (IgG4-RD) is a chronic-fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune disease. Although common organ involvement of IgG4-RD includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established. Recently, five studies identified a subtype of autoimmune hepatitis (AIH), called IgG4-associated AIH (IgG4-AIH). IgG4-AIH is diagnosed based on significant accumulation of IgG4-expressing plasmacytes in the liver in patients who met the diagnostic criteria for classical AIH. Although four of the five reports regarded IgG4-AIH based on hepatic accumulation of IgG4-positive cells alone, one report diagnosed IgG4-AIH based on both hepatic accumulation of IgG4-positive cells and elevated serum concentrations of IgG4. IgG4-AIH diagnosed based on the latter criteria may be a hepatic manifestation of IgG4-RD whereas IgG4-AIH diagnosed based on the former criteria may be a subtype of AIH. In this review article, we summarize and discuss clinicopathological features of IgG4-AIH.

Keywords: Autoimmune hepatitis, IgG4, IgG4-related disease, IgG4-associated autoimmune hepatitis

Core tip: IgG4-associated autoimmune hepatitis (IgG4-AIH) is a new disease entity characterized by the accumulation of IgG4-expressing plasma cells in the liver. Recent studies diagnosed IgG4-AIH based on hepatic accumulation of IgG4+ cells alone or in combination with elevated serum concentrations of IgG4. Further studies are required to determine whether IgG4-AIH is a subtype of AIH or a hepatic manifestation of IgG4-related disease.