Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies

Systemic Sclerosis (SSc) is an autoimmune disease marked by dysregulation of the immune system, tissue fibrosis and dysfunction of the vasculature. Vascular damage, remodeling and inadequate endothelial repair are hallmarks of the disease. Since early stages of SSc, damage and apoptosis of endotheli...

Full description

Bibliographic Details
Main Authors: Djúlio César Zanin-Silva, Maynara Santana-Gonçalves, Marianna Yumi Kawashima-Vasconcelos, Maria Carolina Oliveira
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-12-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2021.788250/full
_version_ 1828138651241938944
author Djúlio César Zanin-Silva
Djúlio César Zanin-Silva
Maynara Santana-Gonçalves
Maynara Santana-Gonçalves
Marianna Yumi Kawashima-Vasconcelos
Marianna Yumi Kawashima-Vasconcelos
Maria Carolina Oliveira
Maria Carolina Oliveira
author_facet Djúlio César Zanin-Silva
Djúlio César Zanin-Silva
Maynara Santana-Gonçalves
Maynara Santana-Gonçalves
Marianna Yumi Kawashima-Vasconcelos
Marianna Yumi Kawashima-Vasconcelos
Maria Carolina Oliveira
Maria Carolina Oliveira
author_sort Djúlio César Zanin-Silva
collection DOAJ
description Systemic Sclerosis (SSc) is an autoimmune disease marked by dysregulation of the immune system, tissue fibrosis and dysfunction of the vasculature. Vascular damage, remodeling and inadequate endothelial repair are hallmarks of the disease. Since early stages of SSc, damage and apoptosis of endothelial cells (ECs) can lead to perivascular inflammation, oxidative stress and tissue hypoxia, resulting in multiple clinical manifestations. Raynaud's phenomenon, edematous puffy hands, digital ulcers, pulmonary artery hypertension, erectile dysfunction, scleroderma renal crisis and heart involvement severely affect quality of life and survival. Understanding pathogenic aspects and biomarkers that reflect endothelial damage in SSc is essential to guide therapeutic interventions. Treatment approaches described for SSc-associated vasculopathy include pharmacological options to improve blood flow and tissue perfusion and, more recently, cellular therapy to enhance endothelial repair, promote angiogenesis and heal injuries. This mini-review examines the current knowledge on cellular and molecular aspects of SSc vasculopathy, as well as established and developing therapeutic approaches for improving the vascular compartment.
first_indexed 2024-04-11T18:39:06Z
format Article
id doaj.art-e644508ce0784d5192c569b916bd92de
institution Directory Open Access Journal
issn 2296-858X
language English
last_indexed 2024-04-11T18:39:06Z
publishDate 2021-12-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Medicine
spelling doaj.art-e644508ce0784d5192c569b916bd92de2022-12-22T04:09:04ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-12-01810.3389/fmed.2021.788250788250Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing StrategiesDjúlio César Zanin-Silva0Djúlio César Zanin-Silva1Maynara Santana-Gonçalves2Maynara Santana-Gonçalves3Marianna Yumi Kawashima-Vasconcelos4Marianna Yumi Kawashima-Vasconcelos5Maria Carolina Oliveira6Maria Carolina Oliveira7Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilBasic and Applied Immunology Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilCenter for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilOncology, Stem Cell and Cell-Therapy Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilCenter for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilInternal Medicine Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilCenter for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilDepartment of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BrazilSystemic Sclerosis (SSc) is an autoimmune disease marked by dysregulation of the immune system, tissue fibrosis and dysfunction of the vasculature. Vascular damage, remodeling and inadequate endothelial repair are hallmarks of the disease. Since early stages of SSc, damage and apoptosis of endothelial cells (ECs) can lead to perivascular inflammation, oxidative stress and tissue hypoxia, resulting in multiple clinical manifestations. Raynaud's phenomenon, edematous puffy hands, digital ulcers, pulmonary artery hypertension, erectile dysfunction, scleroderma renal crisis and heart involvement severely affect quality of life and survival. Understanding pathogenic aspects and biomarkers that reflect endothelial damage in SSc is essential to guide therapeutic interventions. Treatment approaches described for SSc-associated vasculopathy include pharmacological options to improve blood flow and tissue perfusion and, more recently, cellular therapy to enhance endothelial repair, promote angiogenesis and heal injuries. This mini-review examines the current knowledge on cellular and molecular aspects of SSc vasculopathy, as well as established and developing therapeutic approaches for improving the vascular compartment.https://www.frontiersin.org/articles/10.3389/fmed.2021.788250/fullsystemic sclerosisvasculopathycellular therapyendothelial cellsvasodilator agent
spellingShingle Djúlio César Zanin-Silva
Djúlio César Zanin-Silva
Maynara Santana-Gonçalves
Maynara Santana-Gonçalves
Marianna Yumi Kawashima-Vasconcelos
Marianna Yumi Kawashima-Vasconcelos
Maria Carolina Oliveira
Maria Carolina Oliveira
Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
Frontiers in Medicine
systemic sclerosis
vasculopathy
cellular therapy
endothelial cells
vasodilator agent
title Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
title_full Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
title_fullStr Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
title_full_unstemmed Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
title_short Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies
title_sort management of endothelial dysfunction in systemic sclerosis current and developing strategies
topic systemic sclerosis
vasculopathy
cellular therapy
endothelial cells
vasodilator agent
url https://www.frontiersin.org/articles/10.3389/fmed.2021.788250/full
work_keys_str_mv AT djuliocesarzaninsilva managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT djuliocesarzaninsilva managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT maynarasantanagoncalves managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT maynarasantanagoncalves managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT mariannayumikawashimavasconcelos managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT mariannayumikawashimavasconcelos managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT mariacarolinaoliveira managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies
AT mariacarolinaoliveira managementofendothelialdysfunctioninsystemicsclerosiscurrentanddevelopingstrategies