caa a22 4500 445888385 CHVBK 20180317145607.0 cr unu---uuuuu 170323e20110301xx s 000 0 eng 10.1007/s00535-011-0386-x doi (NATIONALLICENCE)springer-10.1007/s00535-011-0386-x Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease [Elektronische Daten] [Kazuichi Okazaki, Kazushige Uchida, Masanori Koyabu, Hideaki Miyoshi, Makoto Takaoka] Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4+plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, patients with type 1 AIP often have extrapancreatic lesions, such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis, showing pathological features similar to those of the pancreatic lesions. Based on these findings, an international concept of and diagnostic criteria for AIP have been proposed recently. Of interest, many synonyms have been proposed for the conditions of AIP and extrapancreatic lesions associated with IgG4, such as "multifocal idiopathic fibrosclerosis,” "IgG4-related autoimmune disease,” "IgG4-related sclerosing disease,” "systemic IgG4-related plasmacytic syndrome (SIPS),” 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 fibrosclerosing disease, in 2009, in which the term "IgG4-related disease” was agreed upon as a minimal consensus to cover 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 diseases. The concept and diagnostic criteria of "IgG4-related disease” will be changed in accordance with future studies. Springer, 2011 IgG4 nationallicence IgG4-related disease nationallicence Autoimmune pancreatitis nationallicence Mikulicz disease nationallicence Regulatory T cell (Treg) nationallicence AIP : Autoimmune pancreatitis nationallicence ANA : Anti-nuclear antibody nationallicence CA-II : Carbonic anhydrase-II nationallicence CTLA-4 : Cytotoxic T lymphocyte antigen-4 nationallicence ERCP : Endoscopic retrograde cholangio-pancreatography nationallicence FCRL : Fc-receptor-like nationallicence IFN-γ : Interferon-γ nationallicence IL-4 : Interleukin-4 nationallicence LF : Lactoferrin nationallicence LPSP : Lymphoplasmacytic sclerosing pancreatitis nationallicence MD : Mikulicz disease nationallicence MHC : Major histocompatibility complex nationallicence MOLPS : Multiorgan lymphoproliferative disease nationallicence PBP : Plasminogen-binding protein nationallicence SjS : Sjögren's syndrome nationallicence PSC : Primary sclerosing cholangitis nationallicence RF : Rheumatoid factor nationallicence SIPS : Systemic IgG4 plasmacytic syndrome nationallicence SLE : Systemic lupus erythematosus nationallicence Treg : Regulatory T cell nationallicence UBR2 : Ubiquitin-protein ligase E3 component n-recognin 2 nationallicence Okazaki Kazuichi Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut Uchida Kazushige Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut Koyabu Masanori Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut Miyoshi Hideaki Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut Takaoka Makoto Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut Journal of Gastroenterology Springer Japan 46/3(2011-03-01), 277-288 0944-1174 46:3<277 2011 46 535 https://doi.org/10.1007/s00535-011-0386-x text/html Onlinezugriff via DOI 1 review-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00535-011-0386-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Okazaki Kazuichi Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut NATIONALLICENCE 700 1- Uchida Kazushige Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut NATIONALLICENCE 700 1- Koyabu Masanori Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut NATIONALLICENCE 700 1- Miyoshi Hideaki Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut NATIONALLICENCE 700 1- Takaoka Makoto Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, 573-1197, Hirakata, Osaka, Japan aut NATIONALLICENCE 773 0- Journal of Gastroenterology Springer Japan 46/3(2011-03-01), 277-288 0944-1174 46:3<277 2011 46 535 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer