Clinical Significance of Immunoglobulin G4-associated Autoimmune Hepatitis

Overview

Journal J Gastroenterol

Specialty Gastroenterology

Date 2010 Sep 24

PMID 20862498

Citations 33

Authors

Takeji Umemura

Yoh Zen

Hideaki Hamano

Satoru Joshita

Tetsuya Ichijo

Kaname Yoshizawa

Kendo Kiyosawa

Masao Ota

Shigeyuki Kawa

Yasuni Nakanuma

Eiji Tanaka

Affiliations

Soon will be listed here.

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.

Citing Articles

Wu S, Wang H Front Immunol. 2023; 14:1278332.

PMID: 37868965 PMC: 10585276. DOI: 10.3389/fimmu.2023.1278332.

IgG4-associated autoimmune hepatitis and cholangitis: A relatively novel entity to consider in cases of seronegative autoimmune hepatitis.

Jutras G, Wong P, Ferreira J, LeBlanc J Can Liver J. 2022; 4(2):99-103.

PMID: 35991763 PMC: 9204940. DOI: 10.3138/canlivj-2020-0023.

Autoimmune Diseases of Digestive Organs-A Multidisciplinary Challenge: A Focus on Hepatopancreatobiliary Manifestation.

Kunovsky L, Dite P, Jabandziev P, Kala Z, Vaculova J, Andrasina T J Clin Med. 2021; 10(24).

PMID: 34945093 PMC: 8705412. DOI: 10.3390/jcm10245796.

Lohr J, Vujasinovic M, Rosendahl J, Stone J, Beuers U Nat Rev Gastroenterol Hepatol. 2021; 19(3):185-197.

PMID: 34750548 DOI: 10.1038/s41575-021-00529-y.

Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report.

Tan Y, Wang J, Chen L World J Clin Cases. 2021; 9(25):7527-7534.

PMID: 34616822 PMC: 8464467. DOI: 10.12998/wjcc.v9.i25.7527.

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