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Review of a novel disease entity, immunoglobulin G4-related disease

Takashi Maehara|Masafumi Moriyama|Seiji Nakamura
대한구강악안면외과학회 (Korean Association Of Oral And Maxillofacial Surgeons)|2020.02|대한구강악안면외과학회지 (Journal of the Korean Association of Oral and Maxillofacial Surgeons)|제46권 제1호|pp. 3-11|DOI 10.5125/jkaoms.2020.46.1.3

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참고문헌

키워드

Immunoglobulin G4-related diseaseT cellMikuliçz’s diseaseKüttner’s tumor
주제분류치의학

초록

Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related disease (IgG4-RD), which has been recognized as a single diagnostic entity for less than 15 years. Histopathologic examination is critical for diagnosis of IgG4-RD. CD4+ T and B cells, including IgG4-expressing plasma cells, constitute the major inflammatory cell populations in IgG4-RD and are thought to cause organ damage and tissue fibrosis. Patients with IgG4-RD who have active, untreated disease exhibit significant increase of IgG4-secreting plasmablasts in the blood. Considerable insight into the immunologic mechanisms of IgG4-RD has been achieved in the last decade using novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploring the in-teractions between CD4+ T cells and B lineage cells is critical for understanding the pathophysiology of IgG4-RD. Establishment of pathogenic T cell clones and identification of antigens specific to these clones constitutes the first steps in determining the pathogenesis of the disease. Herein, the clini-cal features and mechanistic insights regarding pathogenesis of IgG4-RD were reviewed.

Abstract

Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related disease (IgG4-RD), which has been recognized as a single diagnostic entity for less than 15 years. Histopathologic examination is critical for diagnosis of IgG4-RD. CD4+ T and B cells, including IgG4-expressing plasma cells, constitute the major inflammatory cell populations in IgG4-RD and are thought to cause organ damage and tissue fibrosis. Patients with IgG4-RD who have active, untreated disease exhibit significant increase of IgG4-secreting plasmablasts in the blood. Considerable insight into the immunologic mechanisms of IgG4-RD has been achieved in the last decade using novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploring the in-teractions between CD4+ T cells and B lineage cells is critical for understanding the pathophysiology of IgG4-RD. Establishment of pathogenic T cell clones and identification of antigens specific to these clones constitutes the first steps in determining the pathogenesis of the disease. Herein, the clini-cal features and mechanistic insights regarding pathogenesis of IgG4-RD were reviewed.

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관련 논문AI추천

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles신용운, 오영하, 이윤정대한안과학회 · Korean Journal of Ophthalmology2012 · 제26권 제3호Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor곽승기, 김완욱, 민홍기, 박성환, 양석우, 이윤수, Jennifer Lee, Ji Hyeon Ju대한내과학회 · The Korean Journal of Internal Medicine2019 · 제34권 제1호면역글로불린 G4 연관 타액선 및 눈물샘염(Mikulicz 병) 1예박준호, 이경규, 이영민, 조헌포대한이비인후과학회 · 대한이비인후과학회지 두경부외과학2013 · 제56권 제7호악하선에 발생한 면역글로불린 G4 연관 질환 7예박재만, 오욱진, 전태성, 조재구대한이비인후과학회 · 대한이비인후과학회지 두경부외과학2025 · 제68권 제1호Immunoglobulin G4-related sclerosing cholangitisGeorge Goodchild, George Webster, Stephen P. Pereira대한내과학회 · The Korean Journal of Internal Medicine2018 · 제33권 제5호Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease김호성, 배현식, 장기택, 최미순, 최승희, 허진석, 김빛나리, Seokhwi Kim대한병리학회 · Journal of Pathology and Translational Medicine2016 · 제50권 제4호

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