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

Author(s)

Zanin-Silva, Djulio Cesar ^{[1, 2]} ; Santana-Goncalves, Maynara ^{[1, 3]} ; Kawashima-Vasconcelos, Marianna Yumi ^{[1, 4]} ; Oliveira, Maria Carolina ^{[5, 1]}

Affiliation

^[1] Univ Sao Paulo, Ctr Cell Based Therapy, Reg Hemotherapy Ctr, Ribeirao Preto Med Sch, Ribeirao Preto - Brazil

^[2] Univ Sao Paulo, Ribeirao Preto Med Sch, Basic & Appl Immunol Grad Program, Ribeirao Preto - Brazil

^[3] Univ Sao Paulo, Ribeirao Preto Med Sch, Oncology Stem Cell & Cell Therapy Grad Program, Ribeirao Preto - Brazil

^[4] Univ Sao Paulo, Ribeirao Preto Med Sch, Internal Med Grad Program, Ribeirao Preto - Brazil

^[5] Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Internal Med, Ribeirao Preto - Brazil

• Document type: Review article
• Source: FRONTIERS IN MEDICINE; v. 8, DEC 22 2021.
• Web of Science Citations: 0

Abstract

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. (AU)

• FAPESP's process: 17/09420-3 - Evaluation of the impact of hematopoietic stem cell transplantation on the vascular aspects of systemic sclerosis
• Grantee: Marianna Yumi Kawashima Vasconcelos
• Support Opportunities: Scholarships in Brazil - Master
• FAPESP's process: 13/08135-2 - CTC - Center for Cell-Based Therapy
• Grantee: Dimas Tadeu Covas
• Support Opportunities: Research Grants - Research, Innovation and Dissemination Centers - RIDC