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Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis

Systemic sclerosis (SSc) is a severe auto-immune, rheumatic disease, characterized by excessive fibrosis of the skin and visceral organs. SSc is accompanied by high morbidity and mortality rates, and unfortunately, few disease-modifying therapies are currently available. Inflammation, vasculopathy,...

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Autores principales: Papadimitriou, Theodoros-Ioannis, van Caam, Arjan, van der Kraan, Peter M., Thurlings, Rogier M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8869277/
https://www.ncbi.nlm.nih.gov/pubmed/35203525
http://dx.doi.org/10.3390/biomedicines10020316
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author Papadimitriou, Theodoros-Ioannis
van Caam, Arjan
van der Kraan, Peter M.
Thurlings, Rogier M.
author_facet Papadimitriou, Theodoros-Ioannis
van Caam, Arjan
van der Kraan, Peter M.
Thurlings, Rogier M.
author_sort Papadimitriou, Theodoros-Ioannis
collection PubMed
description Systemic sclerosis (SSc) is a severe auto-immune, rheumatic disease, characterized by excessive fibrosis of the skin and visceral organs. SSc is accompanied by high morbidity and mortality rates, and unfortunately, few disease-modifying therapies are currently available. Inflammation, vasculopathy, and fibrosis are the key hallmarks of SSc pathology. In this narrative review, we examine the relationship between inflammation and fibrosis and provide an overview of the efficacy of current and novel treatment options in diminishing SSc-related fibrosis based on selected clinical trials. To do this, we first discuss inflammatory pathways of both the innate and acquired immune systems that are associated with SSc pathophysiology. Secondly, we review evidence supporting the use of first-line therapies in SSc patients. In addition, T cell-, B cell-, and cytokine-specific treatments that have been utilized in SSc are explored. Finally, the potential effectiveness of tyrosine kinase inhibitors and other novel therapeutic approaches in reducing fibrosis is highlighted.
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spelling pubmed-88692772022-02-25 Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis Papadimitriou, Theodoros-Ioannis van Caam, Arjan van der Kraan, Peter M. Thurlings, Rogier M. Biomedicines Review Systemic sclerosis (SSc) is a severe auto-immune, rheumatic disease, characterized by excessive fibrosis of the skin and visceral organs. SSc is accompanied by high morbidity and mortality rates, and unfortunately, few disease-modifying therapies are currently available. Inflammation, vasculopathy, and fibrosis are the key hallmarks of SSc pathology. In this narrative review, we examine the relationship between inflammation and fibrosis and provide an overview of the efficacy of current and novel treatment options in diminishing SSc-related fibrosis based on selected clinical trials. To do this, we first discuss inflammatory pathways of both the innate and acquired immune systems that are associated with SSc pathophysiology. Secondly, we review evidence supporting the use of first-line therapies in SSc patients. In addition, T cell-, B cell-, and cytokine-specific treatments that have been utilized in SSc are explored. Finally, the potential effectiveness of tyrosine kinase inhibitors and other novel therapeutic approaches in reducing fibrosis is highlighted. MDPI 2022-01-29 /pmc/articles/PMC8869277/ /pubmed/35203525 http://dx.doi.org/10.3390/biomedicines10020316 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Papadimitriou, Theodoros-Ioannis
van Caam, Arjan
van der Kraan, Peter M.
Thurlings, Rogier M.
Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title_full Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title_fullStr Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title_full_unstemmed Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title_short Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
title_sort therapeutic options for systemic sclerosis: current and future perspectives in tackling immune-mediated fibrosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8869277/
https://www.ncbi.nlm.nih.gov/pubmed/35203525
http://dx.doi.org/10.3390/biomedicines10020316
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