The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease
IgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obli...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-07-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/21/14/5082 |
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| author | Song-Chou Hsieh Chieh-Yu Shen Hsien-Tzung Liao Ming-Han Chen Cheng-Han Wu Ko-Jen Li Cheng-Shiun Lu Yu-Min Kuo Hung-Cheng Tsai Chang-Youh Tsai Chia-Li Yu |
| author_facet | Song-Chou Hsieh Chieh-Yu Shen Hsien-Tzung Liao Ming-Han Chen Cheng-Han Wu Ko-Jen Li Cheng-Shiun Lu Yu-Min Kuo Hung-Cheng Tsai Chang-Youh Tsai Chia-Li Yu |
| author_sort | Song-Chou Hsieh |
| collection | DOAJ |
| description | IgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis together with increased in situ infiltration of IgG4 bearing-plasma cells which account for more than 40% of all IgG-producing B cells. IgG4-RD can also be diagnosed based on an elevated serum IgG4 level of more than 110 mg/dL (normal < 86.5 mg/mL in adult) in conjunction with protean clinical manifestations in various organs such as pancreato–hepatobiliary inflammation with/without salivary/lacrimal gland enlargement. In the present review, we briefly discuss the role of genetic predisposition, environmental factors and candidate autoantibodies in the pathogenesis of IgG4-RD. Then, we discuss in detail the immunological paradox of IgG4 antibody, the mechanism of modified Th2 response for IgG4 rather than IgE antibody production and the controversial issues in the allergic reactions of IgG4-RD. Finally, we extensively review the implications of different immune-related cells, cytokines/chemokines/growth factors and Toll-like as well as NOD-like receptors in the pathogenesis of tissue fibro-inflammatory reactions. Our proposals for the future investigations and prospective therapeutic strategies for IgG4-RD are shown in the last part. |
| first_indexed | 2024-03-10T18:23:33Z |
| format | Article |
| id | doaj.art-3d14d059d33a4db0a485905adabdd235 |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-10T18:23:33Z |
| publishDate | 2020-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-3d14d059d33a4db0a485905adabdd2352023-11-20T07:11:52ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-07-012114508210.3390/ijms21145082The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related DiseaseSong-Chou Hsieh0Chieh-Yu Shen1Hsien-Tzung Liao2Ming-Han Chen3Cheng-Han Wu4Ko-Jen Li5Cheng-Shiun Lu6Yu-Min Kuo7Hung-Cheng Tsai8Chang-Youh Tsai9Chia-Li Yu10Division of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Allergy, Immunology & Rheumatology, Taipei Veterans General Hospital & National Yang-Ming University, Taipei 11217, TaiwanDivision of Allergy, Immunology & Rheumatology, Taipei Veterans General Hospital & National Yang-Ming University, Taipei 11217, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanDivision of Allergy, Immunology & Rheumatology, Taipei Veterans General Hospital & National Yang-Ming University, Taipei 11217, TaiwanDivision of Allergy, Immunology & Rheumatology, Taipei Veterans General Hospital & National Yang-Ming University, Taipei 11217, TaiwanDivision of Rheumatology, Immunology & Allergy, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei 10002, TaiwanIgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis together with increased in situ infiltration of IgG4 bearing-plasma cells which account for more than 40% of all IgG-producing B cells. IgG4-RD can also be diagnosed based on an elevated serum IgG4 level of more than 110 mg/dL (normal < 86.5 mg/mL in adult) in conjunction with protean clinical manifestations in various organs such as pancreato–hepatobiliary inflammation with/without salivary/lacrimal gland enlargement. In the present review, we briefly discuss the role of genetic predisposition, environmental factors and candidate autoantibodies in the pathogenesis of IgG4-RD. Then, we discuss in detail the immunological paradox of IgG4 antibody, the mechanism of modified Th2 response for IgG4 rather than IgE antibody production and the controversial issues in the allergic reactions of IgG4-RD. Finally, we extensively review the implications of different immune-related cells, cytokines/chemokines/growth factors and Toll-like as well as NOD-like receptors in the pathogenesis of tissue fibro-inflammatory reactions. Our proposals for the future investigations and prospective therapeutic strategies for IgG4-RD are shown in the last part.https://www.mdpi.com/1422-0067/21/14/5082IgG4-related diseasefibroinflammatory disorderlymphoplasmacytic infiltrationstoriform fibrosisobliterative phlebitismodified Th2 response |
| spellingShingle | Song-Chou Hsieh Chieh-Yu Shen Hsien-Tzung Liao Ming-Han Chen Cheng-Han Wu Ko-Jen Li Cheng-Shiun Lu Yu-Min Kuo Hung-Cheng Tsai Chang-Youh Tsai Chia-Li Yu The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease International Journal of Molecular Sciences IgG4-related disease fibroinflammatory disorder lymphoplasmacytic infiltration storiform fibrosis obliterative phlebitis modified Th2 response |
| title | The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease |
| title_full | The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease |
| title_fullStr | The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease |
| title_full_unstemmed | The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease |
| title_short | The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease |
| title_sort | cellular and molecular bases of allergy inflammation and tissue fibrosis in patients with igg4 related disease |
| topic | IgG4-related disease fibroinflammatory disorder lymphoplasmacytic infiltration storiform fibrosis obliterative phlebitis modified Th2 response |
| url | https://www.mdpi.com/1422-0067/21/14/5082 |
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