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Article

Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity

by
Eduardo Martín-Nares
1,
Gabriela Hernández-Molina
1,
Ángel A. Priego-Ranero
1,
Isela Chan-Campos
1,
Gladys S. Herrera-Noguera
1,
Fidel López-Verdugo
1 and
Janette Furuzawa-Carballeda
1,2,*
1
Department 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, Mexico
2
Department of Experimental Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Col. Belisario Dominguez Sección XVI, Mexico City 14080, Mexico
*
Author to whom correspondence should be addressed.
Cells 2023, 12(4), 670; https://doi.org/10.3390/cells12040670
Submission received: 28 November 2022 / Revised: 19 January 2023 / Accepted: 13 February 2023 / Published: 20 February 2023
(This article belongs to the Special Issue T Cell Responses in Human Health and Disease - Second Edition)
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Abstract

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+ T cells (Th2 subsets), CD4+ cytotoxic T lymphocytes (CD4+CTLs), T helper 9 cells, T follicular helper cells (Tfh; Tfh1/Tfh2/Tfh17/Tf regulatory [Tfr]), Foxp3+ 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+) 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+/IL-4+, CD4+/IL-5+, CD4+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+CTLs, Tfh17, Tfh2, and Tfr and lower M1 cell number. An increased CD4+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.
Keywords: immunoglobulin G4-related disease; phenotype; cytotoxic T lymphocytes; Th2 cells; T follicular helper cells immunoglobulin G4-related disease; phenotype; cytotoxic T lymphocytes; Th2 cells; T follicular helper cells

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MDPI and ACS Style

Martín-Nares, E.; Hernández-Molina, G.; Priego-Ranero, Á.A.; Chan-Campos, I.; Herrera-Noguera, G.S.; López-Verdugo, F.; Furuzawa-Carballeda, J. Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity. Cells 2023, 12, 670. https://doi.org/10.3390/cells12040670

AMA Style

Martín-Nares E, Hernández-Molina G, Priego-Ranero ÁA, Chan-Campos I, Herrera-Noguera GS, López-Verdugo F, Furuzawa-Carballeda J. Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity. Cells. 2023; 12(4):670. https://doi.org/10.3390/cells12040670

Chicago/Turabian Style

Martín-Nares, Eduardo, Gabriela Hernández-Molina, Ángel A. Priego-Ranero, Isela Chan-Campos, Gladys S. Herrera-Noguera, Fidel López-Verdugo, and Janette Furuzawa-Carballeda. 2023. "Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity" Cells 12, no. 4: 670. https://doi.org/10.3390/cells12040670

APA Style

Martín-Nares, E., Hernández-Molina, G., Priego-Ranero, Á. A., Chan-Campos, I., Herrera-Noguera, G. S., López-Verdugo, F., & Furuzawa-Carballeda, J. (2023). Peripheral Immunophenotype in IgG4-Related Disease and Its Association with Clinical Phenotypes and Disease Activity. Cells, 12(4), 670. https://doi.org/10.3390/cells12040670

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