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Clinical significance of immunoglobulin G4-associated autoimmune hepatitis.
MedLine Citation:
PMID:  20862498     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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.
Authors:
Takeji Umemura; Yoh Zen; Hideaki Hamano; Satoru Joshita; Tetsuya Ichijo; Kaname Yoshizawa; Kendo Kiyosawa; Masao Ota; Shigeyuki Kawa; Yasuni Nakanuma; Eiji Tanaka
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-09-23
Journal Detail:
Title:  Journal of gastroenterology     Volume:  46 Suppl 1     ISSN:  1435-5922     ISO Abbreviation:  J. Gastroenterol.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2010-11-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9430794     Medline TA:  J Gastroenterol     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  48-55     Citation Subset:  IM    
Affiliation:
Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan. tumemura@shinshu-u.ac.jp
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