2020年 改訂 IgG4関連疾患包括診断基準―The 2020 Revised Comprehensive Diagnostic（RCD）Criteria for IgG4-RD ―厚生労働省難治性疾患等政策研究事業 IgG4関連疾患の診断基準並びに診療指針の確立を目指す研究班
IgG4関連疾患（IgG4-related disease：IgG4-RD）は，Mikulicz病，自己免疫疾患，間質性腎炎，前立腺炎，後腹膜線維症等多彩な疾患を含む病態で，今世紀に本邦から発信された興味深い疾患である．厚生労働省のIgG4関連疾患研究班は，2011年に世界で初めての診断基準であるIgG4関連疾患包括診断基準を発表した．これにより，この新疾患は広く世界に受け入れられ，世界中から多くの報告がなされた．しかし，実臨床においては，生検組織が得られない組織があること，血清IgG4（immunoglobulin G4）値や組織中IgG4陽性細胞数のカットオフ値に対する感度・特異度等の問題...
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| Published in | 日本内科学会雑誌 Vol. 110; no. 5; pp. 962 - 969 |
|---|---|
| Main Authors | , , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
一般社団法人 日本内科学会
10.05.2021
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0021-5384 1883-2083 |
| DOI | 10.2169/naika.110.962 |
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| Abstract | IgG4関連疾患（IgG4-related disease：IgG4-RD）は，Mikulicz病，自己免疫疾患，間質性腎炎，前立腺炎，後腹膜線維症等多彩な疾患を含む病態で，今世紀に本邦から発信された興味深い疾患である．厚生労働省のIgG4関連疾患研究班は，2011年に世界で初めての診断基準であるIgG4関連疾患包括診断基準を発表した．これにより，この新疾患は広く世界に受け入れられ，世界中から多くの報告がなされた．しかし，実臨床においては，生検組織が得られない組織があること，血清IgG4（immunoglobulin G4）値や組織中IgG4陽性細胞数のカットオフ値に対する感度・特異度等の問題が生じてきた．そのため，厚生労働省IgG4関連疾患研究班は，IgG4関連疾患包括診断基準2011を改訂した，2020年 改訂 IgG4関連疾患包括診断基準を提唱する．改訂基準は，1）臨床的・放射線学的診断，2）血清学的診断，3）病理学的診断の3項目からなる．さらに，改訂された病理学的診断基準は，従来の2項目に加え，花筵様線維化と閉塞性静脈炎を含めた3項目から構成される． |
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| AbstractList | IgG4関連疾患（IgG4-related disease：IgG4-RD）は，Mikulicz病，自己免疫疾患，間質性腎炎，前立腺炎，後腹膜線維症等多彩な疾患を含む病態で，今世紀に本邦から発信された興味深い疾患である．厚生労働省のIgG4関連疾患研究班は，2011年に世界で初めての診断基準であるIgG4関連疾患包括診断基準を発表した．これにより，この新疾患は広く世界に受け入れられ，世界中から多くの報告がなされた．しかし，実臨床においては，生検組織が得られない組織があること，血清IgG4（immunoglobulin G4）値や組織中IgG4陽性細胞数のカットオフ値に対する感度・特異度等の問題が生じてきた．そのため，厚生労働省IgG4関連疾患研究班は，IgG4関連疾患包括診断基準2011を改訂した，2020年 改訂 IgG4関連疾患包括診断基準を提唱する．改訂基準は，1）臨床的・放射線学的診断，2）血清学的診断，3）病理学的診断の3項目からなる．さらに，改訂された病理学的診断基準は，従来の2項目に加え，花筵様線維化と閉塞性静脈炎を含めた3項目から構成される． |
| Author | 高橋, 裕樹 佐藤, 康晴 岡崎, 和一 後藤, 浩 石坂, 信和 赤水, 尚史 厚生労働省難治性疾患等政策研究事業IgG4関連疾患の診断基準並びに診療指針の確立を目指す研究班, IgG4関連疾患包括診断基準改訂ワーキンググループ 松井, 祥子 川野, 充弘 梅原, 久範 川, 茂幸 |
| Author_xml | – sequence: 1 fullname: 梅原, 久範 organization: 市立長浜病院リウマチセンター – sequence: 1 fullname: 石坂, 信和 organization: 大阪医科大学内科学III循環器内科 – sequence: 1 fullname: 岡崎, 和一 organization: 関西医科大学内科学第三講座 – sequence: 1 fullname: 川, 茂幸 organization: 松本歯科大学内科 – sequence: 1 fullname: 厚生労働省難治性疾患等政策研究事業IgG4関連疾患の診断基準並びに診療指針の確立を目指す研究班, IgG4関連疾患包括診断基準改訂ワーキンググループ – sequence: 1 fullname: 後藤, 浩 organization: 東京医科大学眼科学分野 – sequence: 1 fullname: 高橋, 裕樹 organization: 札幌医科大学免疫・リウマチ内科学 – sequence: 1 fullname: 佐藤, 康晴 organization: 岡山大学学術研究院保健学域病理学 – sequence: 1 fullname: 松井, 祥子 organization: 富山大学保健管理センター – sequence: 1 fullname: 川野, 充弘 organization: 金沢大学リウマチ・膠原病内科 – sequence: 1 fullname: 赤水, 尚史 organization: 和歌山県立医科大学リウマチ・膠原病科学講座/隈病院内科 |
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| References | 16) Umehara H, et al: How to diagnose IgG4-related disease. Ann Rheum Dis 76: e46, 2017. 30) Wallace ZS, et al: The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Arthritis Rheumatol. 72: 7-19, 2020. 29) Umehara H, Okazaki K: Comprehensive diagnostic criteria for IgG4-related disease. Umehara H, eds. IgG4-Related Disease. Springer, Tokyo, 2013, 35-39. 9) Shimosegawa T, et al: International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 40: 352-358, 2011. 27) Umehara H, et al: IgG4-related disease. Rheumatology, 7th edition ed. ELSEVIER, Amsterdam, 2018, 1494-1501. 6) Notohara K, et al: Guidance for diagnosing autoimmune pancreatitis with biopsy tissues. Pathol Int 70: 699-711, 2020. 5) Deshpande V, et al: Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25: 1181-1192, 2012. 10) Masaki Y, Umehara H: IgG4-related disease-the diagnostic confusion and how to avoid it. Nihon Rinsho Meneki Gakkai Kaishi 32: 478-483, 2009. 22) Dhall D, et al: Use of immunohistochemistry for IgG4 in the distinction of autoimmune pancreatitis from peritumoral pancreatitis. Hum Pathol 41: 643-652, 2010. 17) Umehara H, et al: Current approach to the diagnosis of IgG4-related disease - combination of comprehensive diagnostic and organ-specific criteria. Mod Rheumatol 27: 381-391, 2017. 14) Matsui S, et al: Proposed diagnostic criteria for IgG4-related respiratory disease. Respir Investig 54: 130-132, 2016. 26) Morselli-Labate AM, Pezzilli R: Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: a systematic literature review and meta-analysis. J Gastroenterol Hepatol 24: 15-36, 2009. 13) Goto H, et al: Diagnostic criteria for IgG4-related ophthalmic disease. Jpn J Ophthalmol 59: 1-7, 2015. 15) Mizushima I, et al: IgG4-related periaortitis/periarteritis: an under-recognized condition that is potentially life-threatening. Mod Rheumatol 29: 240-250, 2019. 20) Sumii Y, et al: Treatment outcomes of IgG4-producing marginal zone B-cell lymphoma: a retrospective case series. Int J Hematol 112: 780-786, 2020. 23) Culver EL, et al: Elevated serum IgG4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective IgG4-related disease UK cohort. Am J Gastroenterol 111: 733-743, 2016. 28) Strehl JD, et al: Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Athol 64: 237-243, 2011. 18) Inoue D, et al: IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 94: e680, 2015. 31) Wallace ZS, et al: The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 79: 77-87, 2020. 7) Wallace ZS, et al: IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients. Arthritis Rheumatol 67: 2466-2475, 2015. 12) Ohara H, et al: Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 19: 536-542, 2012. 11) Kawano M, et al: Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol 15: 615-626, 2011. 4) Umehara H, et al: Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22: 21-30, 2012. 24) Yu KH, et al: Diagnostic performance of serum IgG4 levels in patients with IgG4-related disease. Medicine (Baltimore) 94: e1707, 2015. 1) Hamano H, et al: High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344: 732-738, 2001. 8) Carruthers MN, et al: The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 74: 14-18, 2015. 19) Yamada K, et al: New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther 19: 262, 2017. 25) Lin W, et al: Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford) 54: 1982-1990, 2015. 3) Stone JH, et al: IgG4-related disease. N Engl J Med 366: 539-551, 2012. 21) Khan S, et al: Immunoglobulin G4-related disease manifesting as isolated, typical, and nontypical gastroesophageal lesion: a research of literature review. Digestion 101: 506-521, 2020. 2) Umehara H, et al: A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 22: 1-14, 2012. |
| References_xml | – reference: 11) Kawano M, et al: Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol 15: 615-626, 2011. – reference: 29) Umehara H, Okazaki K: Comprehensive diagnostic criteria for IgG4-related disease. Umehara H, eds. IgG4-Related Disease. Springer, Tokyo, 2013, 35-39. – reference: 18) Inoue D, et al: IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 94: e680, 2015. – reference: 17) Umehara H, et al: Current approach to the diagnosis of IgG4-related disease - combination of comprehensive diagnostic and organ-specific criteria. Mod Rheumatol 27: 381-391, 2017. – reference: 20) Sumii Y, et al: Treatment outcomes of IgG4-producing marginal zone B-cell lymphoma: a retrospective case series. Int J Hematol 112: 780-786, 2020. – reference: 21) Khan S, et al: Immunoglobulin G4-related disease manifesting as isolated, typical, and nontypical gastroesophageal lesion: a research of literature review. Digestion 101: 506-521, 2020. – reference: 15) Mizushima I, et al: IgG4-related periaortitis/periarteritis: an under-recognized condition that is potentially life-threatening. Mod Rheumatol 29: 240-250, 2019. – reference: 6) Notohara K, et al: Guidance for diagnosing autoimmune pancreatitis with biopsy tissues. Pathol Int 70: 699-711, 2020. – reference: 2) Umehara H, et al: A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 22: 1-14, 2012. – reference: 9) Shimosegawa T, et al: International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 40: 352-358, 2011. – reference: 22) Dhall D, et al: Use of immunohistochemistry for IgG4 in the distinction of autoimmune pancreatitis from peritumoral pancreatitis. Hum Pathol 41: 643-652, 2010. – reference: 23) Culver EL, et al: Elevated serum IgG4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective IgG4-related disease UK cohort. Am J Gastroenterol 111: 733-743, 2016. – reference: 1) Hamano H, et al: High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344: 732-738, 2001. – reference: 4) Umehara H, et al: Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22: 21-30, 2012. – reference: 24) Yu KH, et al: Diagnostic performance of serum IgG4 levels in patients with IgG4-related disease. Medicine (Baltimore) 94: e1707, 2015. – reference: 19) Yamada K, et al: New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther 19: 262, 2017. – reference: 7) Wallace ZS, et al: IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients. Arthritis Rheumatol 67: 2466-2475, 2015. – reference: 31) Wallace ZS, et al: The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 79: 77-87, 2020. – reference: 28) Strehl JD, et al: Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Athol 64: 237-243, 2011. – reference: 12) Ohara H, et al: Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 19: 536-542, 2012. – reference: 26) Morselli-Labate AM, Pezzilli R: Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: a systematic literature review and meta-analysis. J Gastroenterol Hepatol 24: 15-36, 2009. – reference: 8) Carruthers MN, et al: The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 74: 14-18, 2015. – reference: 16) Umehara H, et al: How to diagnose IgG4-related disease. Ann Rheum Dis 76: e46, 2017. – reference: 30) Wallace ZS, et al: The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Arthritis Rheumatol. 72: 7-19, 2020. – reference: 10) Masaki Y, Umehara H: IgG4-related disease-the diagnostic confusion and how to avoid it. Nihon Rinsho Meneki Gakkai Kaishi 32: 478-483, 2009. – reference: 27) Umehara H, et al: IgG4-related disease. Rheumatology, 7th edition ed. ELSEVIER, Amsterdam, 2018, 1494-1501. – reference: 14) Matsui S, et al: Proposed diagnostic criteria for IgG4-related respiratory disease. Respir Investig 54: 130-132, 2016. – reference: 25) Lin W, et al: Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford) 54: 1982-1990, 2015. – reference: 3) Stone JH, et al: IgG4-related disease. N Engl J Med 366: 539-551, 2012. – reference: 5) Deshpande V, et al: Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25: 1181-1192, 2012. – reference: 13) Goto H, et al: Diagnostic criteria for IgG4-related ophthalmic disease. Jpn J Ophthalmol 59: 1-7, 2015. |
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| Title | 2020年 改訂 IgG4関連疾患包括診断基準―The 2020 Revised Comprehensive Diagnostic（RCD）Criteria for IgG4-RD ―厚生労働省難治性疾患等政策研究事業 IgG4関連疾患の診断基準並びに診療指針の確立を目指す研究班 |
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