T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends

Systemic sclerosis (SSc) is a chronic progressive autoimmune disease characterized by skin and multiorgan involvement with alterations in both the innate and adaptive immunities. The hallmark of the disease is widespread fibrosis engaging the skin and multiple internal organs, as well as the musculo...

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Autores principales:	Krasimirova E., Kyurkchiev D.
Formato:	article
Lenguaje:	EN
Publicado:	Sciendo 2017
Materias:	systemic sclerosis t helper cells treg cells Medicine R
Acceso en línea:	https://doaj.org/article/19bec91a4dc74b059728dbafab982c6b
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spelling	oai:doaj.org-article:19bec91a4dc74b059728dbafab982c6b2021-12-02T19:11:12ZT Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends0324-175010.1515/amb-2017-0010https://doaj.org/article/19bec91a4dc74b059728dbafab982c6b2017-05-01T00:00:00Zhttps://doi.org/10.1515/amb-2017-0010https://doaj.org/toc/0324-1750Systemic sclerosis (SSc) is a chronic progressive autoimmune disease characterized by skin and multiorgan involvement with alterations in both the innate and adaptive immunities. The hallmark of the disease is widespread fibrosis engaging the skin and multiple internal organs, as well as the musculoskeletal system. There is mounting evidence that T cells are key players in the pathogenesis of scleroderma. The current review discusses the role of the different T helper (Th) lymphocyte subsets in the processes of inflammation and fibrosis, characteristics for the pathogenesis of the disease. Cytokines produced by Th cell populations have a major effect on endothelial cells and fibroblasts in the context of favoring/inhibiting the vasculopathy and the fibrosis spread. The Th2 pro-fibrotic cytokines IL-4 and IL-13 have been shown to induce collagen synthesis by fibroblasts, whereas IFN-γ demonstrates an inhibitory effect. Increased Th17 cells are present in the scleroderma skin infiltrates. The combination of IL-17, IFN-γ and TGF-β levels in CD45RO and CD45RA cells from patients with SSc is useful to distinguish between the limited and the diffuse phenotype of the disease. There are accumulating data for functional and numerical alterations in the Tregs in SSc. High levels of TNF-α which might reduce the suppressive ability of Tregs have been described. According to some studies, the number of Tregs in scleroderma skin biopsies has been decreased against the normal absolute number of Tregs in peripheral blood of the same patients, which suggests suppressed immunomodulatory response. Other studies reported increased frequency of Tregs in peripheral blood of patients with systemic sclerosis and established a correlation with disease activity. The main immunological challenge remains the identification of the trigger of the autoimmune response in SSc, the causes for preferential Th2-type cell responses and the immunological differences between the diffuse and the limited cutaneous form of the disease.Krasimirova E.Kyurkchiev D.Sciendoarticlesystemic sclerosist helper cellstreg cellsMedicineRENActa Medica Bulgarica, Vol 44, Iss 1, Pp 57-63 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	systemic sclerosis t helper cells treg cells Medicine R
spellingShingle	systemic sclerosis t helper cells treg cells Medicine R Krasimirova E. Kyurkchiev D. T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
description	Systemic sclerosis (SSc) is a chronic progressive autoimmune disease characterized by skin and multiorgan involvement with alterations in both the innate and adaptive immunities. The hallmark of the disease is widespread fibrosis engaging the skin and multiple internal organs, as well as the musculoskeletal system. There is mounting evidence that T cells are key players in the pathogenesis of scleroderma. The current review discusses the role of the different T helper (Th) lymphocyte subsets in the processes of inflammation and fibrosis, characteristics for the pathogenesis of the disease. Cytokines produced by Th cell populations have a major effect on endothelial cells and fibroblasts in the context of favoring/inhibiting the vasculopathy and the fibrosis spread. The Th2 pro-fibrotic cytokines IL-4 and IL-13 have been shown to induce collagen synthesis by fibroblasts, whereas IFN-γ demonstrates an inhibitory effect. Increased Th17 cells are present in the scleroderma skin infiltrates. The combination of IL-17, IFN-γ and TGF-β levels in CD45RO and CD45RA cells from patients with SSc is useful to distinguish between the limited and the diffuse phenotype of the disease. There are accumulating data for functional and numerical alterations in the Tregs in SSc. High levels of TNF-α which might reduce the suppressive ability of Tregs have been described. According to some studies, the number of Tregs in scleroderma skin biopsies has been decreased against the normal absolute number of Tregs in peripheral blood of the same patients, which suggests suppressed immunomodulatory response. Other studies reported increased frequency of Tregs in peripheral blood of patients with systemic sclerosis and established a correlation with disease activity. The main immunological challenge remains the identification of the trigger of the autoimmune response in SSc, the causes for preferential Th2-type cell responses and the immunological differences between the diffuse and the limited cutaneous form of the disease.
format	article
author	Krasimirova E. Kyurkchiev D.
author_facet	Krasimirova E. Kyurkchiev D.
author_sort	Krasimirova E.
title	T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
title_short	T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
title_full	T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
title_fullStr	T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
title_full_unstemmed	T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends
title_sort	t helper cells in the immunopathogenesis of systemic sclerosis – current trends
publisher	Sciendo
publishDate	2017
url	https://doaj.org/article/19bec91a4dc74b059728dbafab982c6b
work_keys_str_mv	AT krasimirovae thelpercellsintheimmunopathogenesisofsystemicsclerosiscurrenttrends AT kyurkchievd thelpercellsintheimmunopathogenesisofsystemicsclerosiscurrenttrends
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T Helper Cells in the Immunopathogenesis of Systemic Sclerosis – Current Trends

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