Pathogenesis of Systemic Sclerosis
Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the resul...
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Frontiers Media S.A.
2015
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4459100/ |
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pubmed-44591002015-06-23 Pathogenesis of Systemic Sclerosis Pattanaik, Debendra Brown, Monica Postlethwaite, Bradley C. Postlethwaite, Arnold E. Immunology Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the result of: (1) innate/adaptive immune system abnormalities leading to production of autoantibodies and cell-mediated autoimmunity, (2) microvascular endothelial cell/small vessel fibroproliferative vasculopathy, and (3) fibroblast dysfunction generating excessive accumulation of collagen and other matrix components in skin and internal organs. All three of these processes interact and affect each other. The disease is heterogeneous in its clinical presentation that likely reflects different genetic or triggering factor (i.e., infection or environmental toxin) influences on the immune system, vasculature, and connective tissue cells. The roles played by other ubiquitous molecular entities (such as lysophospholipids, endocannabinoids, and their diverse receptors and vitamin D) in influencing the immune system, vasculature, and connective tissue cells are just beginning to be realized and studied and may provide insights into new therapeutic approaches to treat SSc. Frontiers Media S.A. 2015-06-08 /pmc/articles/PMC4459100/ /pubmed/26106387 http://dx.doi.org/10.3389/fimmu.2015.00272 Text en Copyright © 2015 Pattanaik, Brown, Postlethwaite and Postlethwaite. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
| 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 |
Pattanaik, Debendra Brown, Monica Postlethwaite, Bradley C. Postlethwaite, Arnold E. |
| spellingShingle |
Pattanaik, Debendra Brown, Monica Postlethwaite, Bradley C. Postlethwaite, Arnold E. Pathogenesis of Systemic Sclerosis |
| author_facet |
Pattanaik, Debendra Brown, Monica Postlethwaite, Bradley C. Postlethwaite, Arnold E. |
| author_sort |
Pattanaik, Debendra |
| title |
Pathogenesis of Systemic Sclerosis |
| title_short |
Pathogenesis of Systemic Sclerosis |
| title_full |
Pathogenesis of Systemic Sclerosis |
| title_fullStr |
Pathogenesis of Systemic Sclerosis |
| title_full_unstemmed |
Pathogenesis of Systemic Sclerosis |
| title_sort |
pathogenesis of systemic sclerosis |
| description |
Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the result of: (1) innate/adaptive immune system abnormalities leading to production of autoantibodies and cell-mediated autoimmunity, (2) microvascular endothelial cell/small vessel fibroproliferative vasculopathy, and (3) fibroblast dysfunction generating excessive accumulation of collagen and other matrix components in skin and internal organs. All three of these processes interact and affect each other. The disease is heterogeneous in its clinical presentation that likely reflects different genetic or triggering factor (i.e., infection or environmental toxin) influences on the immune system, vasculature, and connective tissue cells. The roles played by other ubiquitous molecular entities (such as lysophospholipids, endocannabinoids, and their diverse receptors and vitamin D) in influencing the immune system, vasculature, and connective tissue cells are just beginning to be realized and studied and may provide insights into new therapeutic approaches to treat SSc. |
| publisher |
Frontiers Media S.A. |
| publishDate |
2015 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4459100/ |
| _version_ |
1613232832828669952 |