Current perspectives on the immunopathogenesis of systemic sclerosis
Systemic sclerosis (SSc or scleroderma) is a progressive and highly debilitating autoimmune disorder characterized by inflammation, vasculopathy, and extensive fibrosis. SSc is highly heterogeneous in its clinical presentation, extent and severity of skin and internal organ involvement, and clinical...
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Dove Medical Press
2016
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4970639/ |
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pubmed-49706392016-08-15 Current perspectives on the immunopathogenesis of systemic sclerosis Fuschiotti, Patrizia Review Systemic sclerosis (SSc or scleroderma) is a progressive and highly debilitating autoimmune disorder characterized by inflammation, vasculopathy, and extensive fibrosis. SSc is highly heterogeneous in its clinical presentation, extent and severity of skin and internal organ involvement, and clinical course and has the highest fatality rate among connective tissue diseases. While clinical outcomes have improved in recent years, no current therapy is able to reverse or slow the natural progression of SSc, a reflection of its complex pathogenesis. Although activation of the immune system has long been recognized, the mechanisms responsible for the initiation of autoimmunity and the role of immune effector pathways in the pathogenesis of SSc remain incompletely understood. This review summarizes recent progress in disease pathogenesis with particular focus on the immunopathogenetic mechanisms of SSc. Dove Medical Press 2016-04-11 /pmc/articles/PMC4970639/ /pubmed/27529059 http://dx.doi.org/10.2147/ITT.S82037 Text en © 2016 Fuschiotti. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. |
| repository_type |
Open Access Journal |
| institution_category |
Foreign Institution |
| institution |
US National Center for Biotechnology Information |
| building |
NCBI PubMed |
| collection |
Online Access |
| language |
English |
| format |
Online |
| author |
Fuschiotti, Patrizia |
| spellingShingle |
Fuschiotti, Patrizia Current perspectives on the immunopathogenesis of systemic sclerosis |
| author_facet |
Fuschiotti, Patrizia |
| author_sort |
Fuschiotti, Patrizia |
| title |
Current perspectives on the immunopathogenesis of systemic sclerosis |
| title_short |
Current perspectives on the immunopathogenesis of systemic sclerosis |
| title_full |
Current perspectives on the immunopathogenesis of systemic sclerosis |
| title_fullStr |
Current perspectives on the immunopathogenesis of systemic sclerosis |
| title_full_unstemmed |
Current perspectives on the immunopathogenesis of systemic sclerosis |
| title_sort |
current perspectives on the immunopathogenesis of systemic sclerosis |
| description |
Systemic sclerosis (SSc or scleroderma) is a progressive and highly debilitating autoimmune disorder characterized by inflammation, vasculopathy, and extensive fibrosis. SSc is highly heterogeneous in its clinical presentation, extent and severity of skin and internal organ involvement, and clinical course and has the highest fatality rate among connective tissue diseases. While clinical outcomes have improved in recent years, no current therapy is able to reverse or slow the natural progression of SSc, a reflection of its complex pathogenesis. Although activation of the immune system has long been recognized, the mechanisms responsible for the initiation of autoimmunity and the role of immune effector pathways in the pathogenesis of SSc remain incompletely understood. This review summarizes recent progress in disease pathogenesis with particular focus on the immunopathogenetic mechanisms of SSc. |
| publisher |
Dove Medical Press |
| publishDate |
2016 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4970639/ |
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1613619902683283456 |