Cargando…
Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6?
Systemic sclerosis (SS) is a chronic autoimmune disorder, which has both cutaneous and systemic clinical manifestations. The disease pathogenesis includes a triad of manifestations, such as vasculopathy, autoimmunity, and fibrosis. Interleukin-6 (IL-6) has a special role in SS development, both in v...
Autores principales: | , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8869570/ https://www.ncbi.nlm.nih.gov/pubmed/35203527 http://dx.doi.org/10.3390/biomedicines10020318 |
_version_ | 1784656529500143616 |
---|---|
author | Cardoneanu, Anca Burlui, Alexandra Maria Macovei, Luana Andreea Bratoiu, Ioana Richter, Patricia Rezus, Elena |
author_facet | Cardoneanu, Anca Burlui, Alexandra Maria Macovei, Luana Andreea Bratoiu, Ioana Richter, Patricia Rezus, Elena |
author_sort | Cardoneanu, Anca |
collection | PubMed |
description | Systemic sclerosis (SS) is a chronic autoimmune disorder, which has both cutaneous and systemic clinical manifestations. The disease pathogenesis includes a triad of manifestations, such as vasculopathy, autoimmunity, and fibrosis. Interleukin-6 (IL-6) has a special role in SS development, both in vascular damage and in the development of fibrosis. In the early stages, IL-6 participates in vascular endothelial activation and apoptosis, leading to the release of damage-associated molecular patterns (DAMPs), which maintain inflammation and autoimmunity. Moreover, IL-6 plays an important role in the development of fibrotic changes by mediating the transformation of fibroblasts into myofibroblasts. All of these are associated with disabling clinical manifestations, such as skin thickening, pulmonary fibrosis, pulmonary arterial hypertension (PAH), heart failure, and dysphagia. Tocilizumab is a humanized monoclonal antibody that inhibits IL-6 by binding to the specific receptor, thus preventing its proinflammatory and fibrotic actions. Anti-IL-6 therapy with Tocilizumab is a new hope for SS patients, with data from clinical trials supporting the favorable effect, especially on skin and lung damage. |
format | Online Article Text |
id | pubmed-8869570 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-88695702022-02-25 Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? Cardoneanu, Anca Burlui, Alexandra Maria Macovei, Luana Andreea Bratoiu, Ioana Richter, Patricia Rezus, Elena Biomedicines Review Systemic sclerosis (SS) is a chronic autoimmune disorder, which has both cutaneous and systemic clinical manifestations. The disease pathogenesis includes a triad of manifestations, such as vasculopathy, autoimmunity, and fibrosis. Interleukin-6 (IL-6) has a special role in SS development, both in vascular damage and in the development of fibrosis. In the early stages, IL-6 participates in vascular endothelial activation and apoptosis, leading to the release of damage-associated molecular patterns (DAMPs), which maintain inflammation and autoimmunity. Moreover, IL-6 plays an important role in the development of fibrotic changes by mediating the transformation of fibroblasts into myofibroblasts. All of these are associated with disabling clinical manifestations, such as skin thickening, pulmonary fibrosis, pulmonary arterial hypertension (PAH), heart failure, and dysphagia. Tocilizumab is a humanized monoclonal antibody that inhibits IL-6 by binding to the specific receptor, thus preventing its proinflammatory and fibrotic actions. Anti-IL-6 therapy with Tocilizumab is a new hope for SS patients, with data from clinical trials supporting the favorable effect, especially on skin and lung damage. MDPI 2022-01-29 /pmc/articles/PMC8869570/ /pubmed/35203527 http://dx.doi.org/10.3390/biomedicines10020318 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 Cardoneanu, Anca Burlui, Alexandra Maria Macovei, Luana Andreea Bratoiu, Ioana Richter, Patricia Rezus, Elena Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title | Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title_full | Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title_fullStr | Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title_full_unstemmed | Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title_short | Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6? |
title_sort | targeting systemic sclerosis from pathogenic mechanisms to clinical manifestations: why il-6? |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8869570/ https://www.ncbi.nlm.nih.gov/pubmed/35203527 http://dx.doi.org/10.3390/biomedicines10020318 |
work_keys_str_mv | AT cardoneanuanca targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 AT burluialexandramaria targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 AT macoveiluanaandreea targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 AT bratoiuioana targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 AT richterpatricia targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 AT rezuselena targetingsystemicsclerosisfrompathogenicmechanismstoclinicalmanifestationswhyil6 |