Clinical Reviews in Allergy & Immunology

, Volume 41, Issue 2, pp 126–138 | Cite as

Recent Concepts of Autoimmune Pancreatitis and IgG4-Related Disease

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


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.


IgG4 IgG4-related disease Autoimmune pancreatitis Mikulicz disease Regulatory T cell (Treg) 



Autoimmune pancreatitis


Anti-nuclear antibody


Carbonic anhydrase-II


Cytotoxic T lymphocyte antigen-4


Endoscopic retrograde cholangiopancreatography










Lymphoplasmacytic sclerosing pancreatitis


Mikulicz disease


Major histocompatibility complex


Multiorgan lymphoproliferative disease


Plasminogen-binding protein


Sjögren’s syndrome


Primary sclerosing cholangitis


Rheumatoid factor


IgG4-systemic plasmacytic syndrome


Systemic lupus erythematosus


Regulatory T cell


Ubiquitin-protein ligase E3 component n-recognin 2



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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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Kazuichi Okazaki
    • 1
  • Kazushige Uchida
    • 1
  • Hideaki Miyoshi
    • 1
  • Tsukasa Ikeura
    • 1
  • Makoto Takaoka
    • 1
  • Akiyoshi Nishio
    • 1
  1. 1.The Third Department of Internal Medicine, Division of Gastroenterology and HepatologyKansai Medical UniversityHirakataJapan

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