Immune dysregulation in immunoglobulin G4–related disease
(IgG4-RD) is an immune-mediated fibrotic disorder characterized by severe resolution of inflammation and dysregulation of wound healing. IgG4-RD has been considered a unique disease since 2003, and significant progress has been achieved in the understanding of its essential features. The central rol...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2023-12-01
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| Series: | Japanese Dental Science Review |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1882761622000412 |
| _version_ | 1797387846390120448 |
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| author | Takashi Maehara Risako Koga Seiji Nakamura |
| author_facet | Takashi Maehara Risako Koga Seiji Nakamura |
| author_sort | Takashi Maehara |
| collection | DOAJ |
| description | (IgG4-RD) is an immune-mediated fibrotic disorder characterized by severe resolution of inflammation and dysregulation of wound healing. IgG4-RD has been considered a unique disease since 2003, and significant progress has been achieved in the understanding of its essential features. The central role of B cells in IgG4-RD has been demonstrated by the robust clinical responsiveness of IgG4-RD to B cell depletion and the identification of multiple self-antigens that promote B cell expansion. Studies have increasingly revealed critical roles of these B cells and T cells in the pathogenesis of IgG4-RD, and we and other authors further identified CD4+ cytotoxic T lymphocytes as the main tissue-infiltrating CD4+ T cell subset in IgG4-RD tissues. Additionally, T follicular helper cell subsets that play a role in IgG4 isotype switching have been identified. In this review, we discuss research on IgG4-RD and the roles of B cell and T cell subsets, as well as the functions of CD4+ cytotoxic T cells in IgG4-RD pathogenesis. We highlight our findings from ongoing research using single-cell analysis of infiltrating CD4+ cytotoxic T cells, CD4+ follicular helper T cells, and infiltrating B cells in IgG4-RD and propose a model for the pathogenesis of IgG4-RD. |
| first_indexed | 2024-03-08T22:31:00Z |
| format | Article |
| id | doaj.art-10d9c36782b946a4ab13d0b6e9d01eed |
| institution | Directory Open Access Journal |
| issn | 1882-7616 |
| language | English |
| last_indexed | 2024-03-08T22:31:00Z |
| publishDate | 2023-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Japanese Dental Science Review |
| spelling | doaj.art-10d9c36782b946a4ab13d0b6e9d01eed2023-12-18T04:24:03ZengElsevierJapanese Dental Science Review1882-76162023-12-015917Immune dysregulation in immunoglobulin G4–related diseaseTakashi Maehara0Risako Koga1Seiji Nakamura2Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan; Dento-craniofacial Development and Regeneration Research Center, Faculty of Dental Science, Kyushu University, Fukuoka, Japan; Correspondence to: Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, 3–1-1 Maidashi, Higashi-ku, Fukuoka 812–8582, Japan.Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, JapanSection of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan(IgG4-RD) is an immune-mediated fibrotic disorder characterized by severe resolution of inflammation and dysregulation of wound healing. IgG4-RD has been considered a unique disease since 2003, and significant progress has been achieved in the understanding of its essential features. The central role of B cells in IgG4-RD has been demonstrated by the robust clinical responsiveness of IgG4-RD to B cell depletion and the identification of multiple self-antigens that promote B cell expansion. Studies have increasingly revealed critical roles of these B cells and T cells in the pathogenesis of IgG4-RD, and we and other authors further identified CD4+ cytotoxic T lymphocytes as the main tissue-infiltrating CD4+ T cell subset in IgG4-RD tissues. Additionally, T follicular helper cell subsets that play a role in IgG4 isotype switching have been identified. In this review, we discuss research on IgG4-RD and the roles of B cell and T cell subsets, as well as the functions of CD4+ cytotoxic T cells in IgG4-RD pathogenesis. We highlight our findings from ongoing research using single-cell analysis of infiltrating CD4+ cytotoxic T cells, CD4+ follicular helper T cells, and infiltrating B cells in IgG4-RD and propose a model for the pathogenesis of IgG4-RD.http://www.sciencedirect.com/science/article/pii/S1882761622000412AutoimmuneAnti-inflammatorySingle-cell RNA sequencingIgG4-related diseaseCytotoxic T cellsSalivary gland |
| spellingShingle | Takashi Maehara Risako Koga Seiji Nakamura Immune dysregulation in immunoglobulin G4–related disease Japanese Dental Science Review Autoimmune Anti-inflammatory Single-cell RNA sequencing IgG4-related disease Cytotoxic T cells Salivary gland |
| title | Immune dysregulation in immunoglobulin G4–related disease |
| title_full | Immune dysregulation in immunoglobulin G4–related disease |
| title_fullStr | Immune dysregulation in immunoglobulin G4–related disease |
| title_full_unstemmed | Immune dysregulation in immunoglobulin G4–related disease |
| title_short | Immune dysregulation in immunoglobulin G4–related disease |
| title_sort | immune dysregulation in immunoglobulin g4 related disease |
| topic | Autoimmune Anti-inflammatory Single-cell RNA sequencing IgG4-related disease Cytotoxic T cells Salivary gland |
| url | http://www.sciencedirect.com/science/article/pii/S1882761622000412 |
| work_keys_str_mv | AT takashimaehara immunedysregulationinimmunoglobuling4relateddisease AT risakokoga immunedysregulationinimmunoglobuling4relateddisease AT seijinakamura immunedysregulationinimmunoglobuling4relateddisease |