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Recent Concepts of Autoimmune Pancreatitis and IgG4-Related Disease

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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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Abbreviations

AIP:

Autoimmune pancreatitis

ANA:

Anti-nuclear antibody

CA-II:

Carbonic anhydrase-II

CTLA-4 :

Cytotoxic T lymphocyte antigen-4

ERCP:

Endoscopic retrograde cholangiopancreatography

FCRL:

Fc-receptor-like

IFN-γ:

Interferon-γ

IL-4:

Interleukin-4

LF:

Lactoferrin

LPSP:

Lymphoplasmacytic sclerosing pancreatitis

MD:

Mikulicz disease

MHC:

Major histocompatibility complex

MOLPS:

Multiorgan lymphoproliferative disease

PBP:

Plasminogen-binding protein

SjS:

Sjögren’s syndrome

PSC:

Primary sclerosing cholangitis

RF:

Rheumatoid factor

SIPS:

IgG4-systemic plasmacytic syndrome

SLE:

Systemic lupus erythematosus

Treg:

Regulatory T cell

UBR2:

Ubiquitin-protein ligase E3 component n-recognin 2

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Acknowledgment

This study was partly supported by a Grant-in-Aid for Scientific Research from the Ministry of Culture and Science of Japan (20590810) and a Grant-in-Aid for “Research for Intractable Disease” Program from the Ministry of Health, Labor and Welfare of Japan.

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  1. The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan

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

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  1. Kazuichi Okazaki
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Correspondence to Kazuichi Okazaki.

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Okazaki, K., Uchida, K., Miyoshi, H. et al. Recent Concepts of Autoimmune Pancreatitis and IgG4-Related Disease. Clinic Rev Allerg Immunol 41, 126–138 (2011). https://doi.org/10.1007/s12016-010-8214-2

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