IgG4関連硬化性胆管炎におけるトピックス
IgG4硬化性胆管炎（IgG4-SC）はIgG4関連疾患の胆道病変と考えられている．診断は画像所見，高IgG4血症，胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に基づくIgG4-SC臨床診断基準2012で可能である．IgG4-SCは時に原発性硬化性胆管炎（PSC）あるいは胆管癌との鑑別診断が困難な場合がある．最近IgG4-SCの診療ガイドラインが作成された．胆管像分類は鑑別診断に有用である．下部胆管狭窄では胆管癌および膵癌を鑑別する．肝門部領域の狭窄を示す場合には，PSC，胆管癌および濾胞性胆管炎を鑑別する．治療はステロイド治療が標準治療であり，経口プレドニゾロン0.6mg/kg...
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| Published in | 胆道 Vol. 33; no. 1; pp. 22 - 31 |
|---|---|
| Main Author | |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本胆道学会
25.03.2019
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0914-0077 1883-6879 |
| DOI | 10.11210/tando.33.22 |
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| Abstract | IgG4硬化性胆管炎（IgG4-SC）はIgG4関連疾患の胆道病変と考えられている．診断は画像所見，高IgG4血症，胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に基づくIgG4-SC臨床診断基準2012で可能である．IgG4-SCは時に原発性硬化性胆管炎（PSC）あるいは胆管癌との鑑別診断が困難な場合がある．最近IgG4-SCの診療ガイドラインが作成された．胆管像分類は鑑別診断に有用である．下部胆管狭窄では胆管癌および膵癌を鑑別する．肝門部領域の狭窄を示す場合には，PSC，胆管癌および濾胞性胆管炎を鑑別する．治療はステロイド治療が標準治療であり，経口プレドニゾロン0.6mg/kg/日を2～4週間投与後漸減する．ほとんどの場合ステロイド治療が奏功するが，ステロイド治療後胆管像に程度の差があるが狭窄が残存することもある．予後はおおむね良好と考えられるが，長期予後は不明である． |
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| AbstractList | 「要旨:」IgG4硬化性胆管炎(IgG4-SC)はIgG4関連疾患の胆道病変と考えられている. 診断は画像所見, 高IgG4血症, 胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に基づくIgG4-SC臨床診断基準2012で可能である. IgG4-SCは時に原発性硬化性胆管炎(PSC)あるいは胆管癌との鑑別診断が困難な場合がある. 最近IgG4-SCの診療ガイドラインが作成された. 胆管像分類は鑑別診断に有用である. 下部胆管狭窄では胆管癌および膵癌を鑑別する. 肝門部領域の狭窄を示す場合には, PSC, 胆管癌および濾胞性胆管炎を鑑別する. 治療はステロイド治療が標準治療であり, 経口プレドニゾロン0.6mg/kg/日を2～4週間投与後漸減する. ほとんどの場合ステロイド治療が奏功するが, ステロイド治療後胆管像に程度の差があるが狭窄が残存することもある. 予後はおおむね良好と考えられるが, 長期予後は不明である. IgG4硬化性胆管炎（IgG4-SC）はIgG4関連疾患の胆道病変と考えられている．診断は画像所見，高IgG4血症，胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に基づくIgG4-SC臨床診断基準2012で可能である．IgG4-SCは時に原発性硬化性胆管炎（PSC）あるいは胆管癌との鑑別診断が困難な場合がある．最近IgG4-SCの診療ガイドラインが作成された．胆管像分類は鑑別診断に有用である．下部胆管狭窄では胆管癌および膵癌を鑑別する．肝門部領域の狭窄を示す場合には，PSC，胆管癌および濾胞性胆管炎を鑑別する．治療はステロイド治療が標準治療であり，経口プレドニゾロン0.6mg/kg/日を2～4週間投与後漸減する．ほとんどの場合ステロイド治療が奏功するが，ステロイド治療後胆管像に程度の差があるが狭窄が残存することもある．予後はおおむね良好と考えられるが，長期予後は不明である． |
| Author | 西野, 隆義 |
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| DOI | 10.11210/tando.33.22 |
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| References | 8) Nakazawa T, Naitoh I, Hayashi K, et al. Diagnosis of IgG4-related sclerosing cholangitis. Word J Gastroenterol 2013; 19: 7661-7670 11) Umemura T, Ota M, Hamano H, et al. Association of Autoimmune pancreatitis with Cytotoxic T-lymphocyte Antigen Gene Polymorphisms in Japanese Patients. Am J Gastroenterol 2008; 103: 588-594 10) Kawa S, Ota M, Yoashizawa K, et al. HLA DRB1＊0405-DQB1＊0401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology 2002; 122: 1264-1269 16) Tanaka A, Tazuma S, Okazaki K, et al. Clinical Features, Response to Treatment, and outcomes of IgG4-related Sclerosing Cholangitis. Clinical Gastroenterol and Hepatology 2017; 15: 920-926 19) Ohara H, Nakazawa T, Kawa S, et al. Establishment of a serum IgG4 cut-off value for differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort. J Gastroenterol Hepatol 2013; 28: 1247-1251 29) Graham R, Smyrk T, Chari S, et al. Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. Hum Pathol 2014; 45: 1722-1729 28) Zen Y, Ishikawa A, Ogiso S, et al. Follicular cholangitis and pancreatitis-clinicopathological features and differential diagnosis of an under-recognized entity. Histopathology 2012; 60: 261-269 9) Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci 2019; 26: 9-42 25) Nakazawa T, Naitoh I, Hayashi K, et al. Diagnostic citeria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol 2012; 47: 79-87 24) Itoi T, Kamisawa T, Igarashi Y, et al. The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis. J Gastroenterol 2013; 48: 504-514 27) Kawakami H, Zen Y, Kuwatani M, et al. IgG4-related sclerosing cholangitis and autoimmune pancreatitis: Histological assessment of biopsies from Vater's ampulla and bile duct. J Gastroenterol Hepatol 2010; 25: 1648-1655 32) Masamune A, Nishimori I, Kikuta K, et al. Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. Gut 2017; 66: 487-494 26) Naitoh I, Nakazawa T, Ohara H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis. J Gastroenterol 2009; 44: 1147-1155 7) Zen Y, Kawakami H, Kim JH. IgG4-related sclerosing cholangitis: all we need to know. J Gastroenterol 2016; 51: 295-312 30) Nakazawa T, Ikeda Y, Kawaguchi Y, et al. Isolated inrapancreatic IgG4-related sclerosing cholangitis. Word J Gastreoneterol 2015; 21: 1334-1343 33) Nishino T, Toki F, Oyama H, et al. Biliary tract involvement in autoimmune pancreatitis. Pancreas 2005; 30: 76-82 5) 厚生労働省IgG4関連全身硬化性疾患の診断法の確立と治療方法の開発に関する研究班, 厚生労働省難治性の肝胆道疾患に関する調査研究班, 日本胆道学会. IgG4関連硬化性胆管炎臨床診断基準2012. 胆道 2012; 26: 59-63 34) Tomiyama T, Uchida K, Matsushita M, et al. Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis. J Gastroenterol 2011; 46: 696-704 12) Yanagawa M, Uchida K, Ando Y, et al. Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. J Gastroenterol 2018; 53: 449-460 14) Zen Y, Britton D, Mitra V, et al. A global proteomic study indetifies distinct pathological features of IgG4-related and primary sclerosing cholangitis. Histopathology 2016; 68: 796-809 13) Zen Y, Fujii T, Harada K, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4- related sclerosing pancreatitis and cholangitis. Hepatology 2007; 45: 1538-1546 15) Shiokawa M, Kodama Y, Sekiguchi K, et al: Laminin 511 is a target antigen in autoimmune pancreatitis. Sci Trans Med 2018; 201; 453, Pii: essq0997 18) Naitoh I, Nakazawa T, Ohara H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related scerosing cholangitis. J Gastroenterol 2009; 44: 1147-1155 2) Stone JH, Khosroshahi A, Deshpande V, et al. Recomendations for nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 2012; 64: 3061-3067 6) Ohara H, Okazaki K, Tsubouchi H, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012; 19: 536-542 17) Kanno A, Masamune A, Okazaki K, et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas 2015; 44: 535-539 3) Kamisawa T, Zen Y, Nakazawa T. Advances in IgG4-related pancreatobiliary disease. Lancet Gastroenterol hepatol 2018; 3: 575-585 22) Nakazawa T, Ohara H, Sano H, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointestinal Endosc 2004; 60: 937-944 31) Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut 2009; 58: 1504-1507 4) Okazaki K, Uchida K. Current concept of autoimmune pancreaitis and IgG4-related disease. Am J Gastroenterol 2018; 113: 1412-1416 1) Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Modern Rheumatol 2012; 22: 1-14 23) Nishino T, Oyama H, Hashimoto E, et al. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastoenterol 2007; 42: 550-559 20) Detlefsten S, Kloppel G. IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. Virchow Arch 2018; 472: 545-556 21) 西野隆義, 山岸直子, 橋本悦子, ほか. 原発性硬化性胆管炎. 日本臨床 2012; 70 Suppl 1 : 628-632 |
| References_xml | – reference: 1) Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Modern Rheumatol 2012; 22: 1-14 – reference: 6) Ohara H, Okazaki K, Tsubouchi H, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012; 19: 536-542 – reference: 33) Nishino T, Toki F, Oyama H, et al. Biliary tract involvement in autoimmune pancreatitis. Pancreas 2005; 30: 76-82 – reference: 3) Kamisawa T, Zen Y, Nakazawa T. Advances in IgG4-related pancreatobiliary disease. Lancet Gastroenterol hepatol 2018; 3: 575-585 – reference: 5) 厚生労働省IgG4関連全身硬化性疾患の診断法の確立と治療方法の開発に関する研究班, 厚生労働省難治性の肝胆道疾患に関する調査研究班, 日本胆道学会. IgG4関連硬化性胆管炎臨床診断基準2012. 胆道 2012; 26: 59-63 – reference: 11) Umemura T, Ota M, Hamano H, et al. Association of Autoimmune pancreatitis with Cytotoxic T-lymphocyte Antigen Gene Polymorphisms in Japanese Patients. Am J Gastroenterol 2008; 103: 588-594 – reference: 7) Zen Y, Kawakami H, Kim JH. IgG4-related sclerosing cholangitis: all we need to know. J Gastroenterol 2016; 51: 295-312 – reference: 25) Nakazawa T, Naitoh I, Hayashi K, et al. Diagnostic citeria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol 2012; 47: 79-87 – reference: 9) Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci 2019; 26: 9-42 – reference: 24) Itoi T, Kamisawa T, Igarashi Y, et al. The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis. J Gastroenterol 2013; 48: 504-514 – reference: 10) Kawa S, Ota M, Yoashizawa K, et al. HLA DRB1＊0405-DQB1＊0401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology 2002; 122: 1264-1269 – reference: 28) Zen Y, Ishikawa A, Ogiso S, et al. Follicular cholangitis and pancreatitis-clinicopathological features and differential diagnosis of an under-recognized entity. Histopathology 2012; 60: 261-269 – reference: 26) Naitoh I, Nakazawa T, Ohara H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis. J Gastroenterol 2009; 44: 1147-1155 – reference: 21) 西野隆義, 山岸直子, 橋本悦子, ほか. 原発性硬化性胆管炎. 日本臨床 2012; 70 Suppl 1 : 628-632 – reference: 17) Kanno A, Masamune A, Okazaki K, et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas 2015; 44: 535-539 – reference: 16) Tanaka A, Tazuma S, Okazaki K, et al. Clinical Features, Response to Treatment, and outcomes of IgG4-related Sclerosing Cholangitis. Clinical Gastroenterol and Hepatology 2017; 15: 920-926 – reference: 22) Nakazawa T, Ohara H, Sano H, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointestinal Endosc 2004; 60: 937-944 – reference: 23) Nishino T, Oyama H, Hashimoto E, et al. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastoenterol 2007; 42: 550-559 – reference: 4) Okazaki K, Uchida K. Current concept of autoimmune pancreaitis and IgG4-related disease. Am J Gastroenterol 2018; 113: 1412-1416 – reference: 32) Masamune A, Nishimori I, Kikuta K, et al. Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. Gut 2017; 66: 487-494 – reference: 18) Naitoh I, Nakazawa T, Ohara H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related scerosing cholangitis. J Gastroenterol 2009; 44: 1147-1155 – reference: 29) Graham R, Smyrk T, Chari S, et al. Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. Hum Pathol 2014; 45: 1722-1729 – reference: 31) Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut 2009; 58: 1504-1507 – reference: 15) Shiokawa M, Kodama Y, Sekiguchi K, et al: Laminin 511 is a target antigen in autoimmune pancreatitis. Sci Trans Med 2018; 201; 453, Pii: essq0997 – reference: 19) Ohara H, Nakazawa T, Kawa S, et al. Establishment of a serum IgG4 cut-off value for differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort. J Gastroenterol Hepatol 2013; 28: 1247-1251 – reference: 8) Nakazawa T, Naitoh I, Hayashi K, et al. Diagnosis of IgG4-related sclerosing cholangitis. Word J Gastroenterol 2013; 19: 7661-7670 – reference: 20) Detlefsten S, Kloppel G. IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. Virchow Arch 2018; 472: 545-556 – reference: 2) Stone JH, Khosroshahi A, Deshpande V, et al. Recomendations for nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 2012; 64: 3061-3067 – reference: 12) Yanagawa M, Uchida K, Ando Y, et al. Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. J Gastroenterol 2018; 53: 449-460 – reference: 27) Kawakami H, Zen Y, Kuwatani M, et al. IgG4-related sclerosing cholangitis and autoimmune pancreatitis: Histological assessment of biopsies from Vater's ampulla and bile duct. J Gastroenterol Hepatol 2010; 25: 1648-1655 – reference: 30) Nakazawa T, Ikeda Y, Kawaguchi Y, et al. Isolated inrapancreatic IgG4-related sclerosing cholangitis. Word J Gastreoneterol 2015; 21: 1334-1343 – reference: 34) Tomiyama T, Uchida K, Matsushita M, et al. Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis. J Gastroenterol 2011; 46: 696-704 – reference: 13) Zen Y, Fujii T, Harada K, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4- related sclerosing pancreatitis and cholangitis. Hepatology 2007; 45: 1538-1546 – reference: 14) Zen Y, Britton D, Mitra V, et al. A global proteomic study indetifies distinct pathological features of IgG4-related and primary sclerosing cholangitis. Histopathology 2016; 68: 796-809 |
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| Snippet | IgG4硬化性胆管炎（IgG4-SC）はIgG4関連疾患の胆道病変と考えられている．診断は画像所見，高IgG4血症，胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に... 「要旨:」IgG4硬化性胆管炎(IgG4-SC)はIgG4関連疾患の胆道病変と考えられている. 診断は画像所見, 高IgG4血症, 胆管外のIgG4関連疾患の存在および胆管壁の病理組織学所見に... |
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| SubjectTerms | IgG4関連硬化性胆管炎 ステロイド治療 原発性硬化性胆管炎 胆管癌 自己免疫性膵炎 |
| Title | IgG4関連硬化性胆管炎におけるトピックス |
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