Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity
Diverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity status is not known. Levels of IL-4-, IL-13-, IL-5-, and IL-21-producing CD4<sup>+</sup> T cells (Th2 subsets), CD4<s...
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2023-02-01
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author | Eduardo Martín-Nares Gabriela Hernández-Molina Ángel A. Priego-Ranero Isela Chan-Campos Gladys S. Herrera-Noguera Fidel López-Verdugo Janette Furuzawa-Carballeda |
author_facet | Eduardo Martín-Nares Gabriela Hernández-Molina Ángel A. Priego-Ranero Isela Chan-Campos Gladys S. Herrera-Noguera Fidel López-Verdugo Janette Furuzawa-Carballeda |
author_sort | Eduardo Martín-Nares |
collection | DOAJ |
description | Diverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity status is not known. Levels of IL-4-, IL-13-, IL-5-, and IL-21-producing CD4<sup>+</sup> T cells (Th2 subsets), CD4<sup>+</sup> cytotoxic T lymphocytes (CD4<sup>+</sup>CTLs), T helper 9 cells, T follicular helper cells (Tfh; Tfh1/Tfh2/Tfh17/Tf regulatory [Tfr]), Foxp3<sup>+</sup> regulatory T cells, Type 1 regulatory T cells (Tr1), T helper 3 regulatory cells (Th3), IL-10-producing regulatory B cells (Bregs), IL-10-expressing regulatory plasmacytoid dendritic (pDC IL-10<sup>+</sup>) cells, and M1 and M2 monocytes were determined by flow cytometry in 43 IgG4-RD patients and 12 controls. All immune subsets were higher in patients vs. controls. CD4<sup>+</sup>/IL-4<sup>+</sup>, CD4<sup>+</sup>/IL-5<sup>+</sup>, CD4<sup>+</sup>CTLs, Tfh2, Tfh17, Tfr, and M1 monocyte cell number was different among IgG4-RD clinical phenotypes. The pancreato-hepato-biliary phenotype was characterized by a higher CD4<sup>+</sup>CTLs, Tfh17, Tfh2, and Tfr and lower M1 cell number. An increased CD4<sup>+</sup>CTLs and Th3 cell number distinguished the head and neck-limited phenotype, while the retroperitoneal/aortic and Mikulicz/systemic phenotypes were characterized by increased Th2 subsets. Tfh17, Tr1, Th3, pDC, M1, and M2 monocytes were augmented in active patients. In summary, the clinical heterogeneity of IgG4-RD might be driven by the participation of different immunophenotypes and, consequently, by a different fibroinflammatory process. |
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spelling | doaj.art-d478d1abe4304572b925f8ed9b08208c2023-11-16T19:45:37ZengMDPI AGCells2073-44092023-02-0112467010.3390/cells12040670Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease ActivityEduardo Martín-Nares0Gabriela Hernández-Molina1Ángel A. Priego-Ranero2Isela Chan-Campos3Gladys S. Herrera-Noguera4Fidel López-Verdugo5Janette Furuzawa-Carballeda6Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, MexicoDiverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity status is not known. Levels of IL-4-, IL-13-, IL-5-, and IL-21-producing CD4<sup>+</sup> T cells (Th2 subsets), CD4<sup>+</sup> cytotoxic T lymphocytes (CD4<sup>+</sup>CTLs), T helper 9 cells, T follicular helper cells (Tfh; Tfh1/Tfh2/Tfh17/Tf regulatory [Tfr]), Foxp3<sup>+</sup> regulatory T cells, Type 1 regulatory T cells (Tr1), T helper 3 regulatory cells (Th3), IL-10-producing regulatory B cells (Bregs), IL-10-expressing regulatory plasmacytoid dendritic (pDC IL-10<sup>+</sup>) cells, and M1 and M2 monocytes were determined by flow cytometry in 43 IgG4-RD patients and 12 controls. All immune subsets were higher in patients vs. controls. CD4<sup>+</sup>/IL-4<sup>+</sup>, CD4<sup>+</sup>/IL-5<sup>+</sup>, CD4<sup>+</sup>CTLs, Tfh2, Tfh17, Tfr, and M1 monocyte cell number was different among IgG4-RD clinical phenotypes. The pancreato-hepato-biliary phenotype was characterized by a higher CD4<sup>+</sup>CTLs, Tfh17, Tfh2, and Tfr and lower M1 cell number. An increased CD4<sup>+</sup>CTLs and Th3 cell number distinguished the head and neck-limited phenotype, while the retroperitoneal/aortic and Mikulicz/systemic phenotypes were characterized by increased Th2 subsets. Tfh17, Tr1, Th3, pDC, M1, and M2 monocytes were augmented in active patients. In summary, the clinical heterogeneity of IgG4-RD might be driven by the participation of different immunophenotypes and, consequently, by a different fibroinflammatory process.https://www.mdpi.com/2073-4409/12/4/670immunoglobulin G4-related diseasephenotypecytotoxic T lymphocytesTh2 cellsT follicular helper cells |
spellingShingle | Eduardo Martín-Nares Gabriela Hernández-Molina Ángel A. Priego-Ranero Isela Chan-Campos Gladys S. Herrera-Noguera Fidel López-Verdugo Janette Furuzawa-Carballeda Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity Cells immunoglobulin G4-related disease phenotype cytotoxic T lymphocytes Th2 cells T follicular helper cells |
title | Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity |
title_full | Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity |
title_fullStr | Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity |
title_full_unstemmed | Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity |
title_short | Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity |
title_sort | peripheral immunophenotype in igg4 related disease and its association with clinical phenotypes and disease activity |
topic | immunoglobulin G4-related disease phenotype cytotoxic T lymphocytes Th2 cells T follicular helper cells |
url | https://www.mdpi.com/2073-4409/12/4/670 |
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