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Clinical Reviews in Allergy & Immunology
Published in: Clinical Reviews in Allergy & Immunology 2/2011

01-10-2011

Recent Concepts of Autoimmune Pancreatitis and IgG4-Related Disease

Authors: Kazuichi Okazaki, Kazushige Uchida, Hideaki Miyoshi, Tsukasa Ikeura, Makoto Takaoka, Akiyoshi Nishio

Published in: Clinical Reviews in Allergy & Immunology | Issue 2/2011

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Abstract

Recent studies suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 related with IgG4 (lymphoplasmacytic sclerosing pancreatitis; LPSP) and type 2 related with a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis; IDCP). Apart from type 2 AIP, the pathological features of type 1 AIP with increased serum IgG4/IgE levels, abundant infiltration of IgG4+ plasmacytes and lymphocytes, fibrosis, and steroid responsiveness are suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, the patients with type 1 AIP often have extrapancreatic lesions such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis showing similar pathological features. Based on these findings, many synonyms have been proposed for these conditions, such as “multifocal idiopathic fibrosclerosis”, “IgG4-related autoimmune disease”, “IgG4-related sclerosing disease”, “IgG4-related plasmacytic disease”, and “IgG4-related multiorgan lymphoproliferative syndrome”, all of which may refer to the same conditions. Therefore, the Japanese Research Committee for “Systemic IgG4-related Sclerosing Disease” proposed a disease concept and clinical diagnostic criteria based on the concept of multifocal fibrosclerosis in 2009, in which the term “IgG4-related disease” was appointed as a minimal consensus on these conditions. Although the significance of IgG4 in the development of “IgG4-related disease” remains unclear, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-related disease. The concept and diagnostic criteria of “IgG4-related disease” will be changed in accordance with future studies.
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Metadata
Title
Recent Concepts of Autoimmune Pancreatitis and IgG4-Related Disease
Authors
Kazuichi Okazaki
Kazushige Uchida
Hideaki Miyoshi
Tsukasa Ikeura
Makoto Takaoka
Akiyoshi Nishio
Publication date
01-10-2011
Publisher
Humana Press Inc
Published in
Clinical Reviews in Allergy & Immunology / Issue 2/2011
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-010-8214-2

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