Zanin-Silva Djúlio César, Santana-Gonçalves Maynara, Kawashima-Vasconcelos Marianna Yumi, Oliveira Maria Carolina
Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
Basic and Applied Immunology Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
Front Med (Lausanne). 2021 Dec 22;8:788250. doi: 10.3389/fmed.2021.788250. eCollection 2021.
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.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为免疫系统失调、组织纤维化和血管系统功能障碍。血管损伤、重塑以及内皮修复不足是该疾病的标志。自SSc早期起，内皮细胞（ECs）的损伤和凋亡可导致血管周围炎症、氧化应激和组织缺氧，从而引发多种临床表现。雷诺现象、手部水肿、指端溃疡、肺动脉高压、勃起功能障碍、硬皮病肾危象和心脏受累严重影响生活质量和生存率。了解SSc中反映内皮损伤的致病因素和生物标志物对于指导治疗干预至关重要。针对SSc相关血管病变描述的治疗方法包括改善血流和组织灌注的药物选择，以及最近用于增强内皮修复、促进血管生成和愈合损伤的细胞疗法。本综述探讨了关于SSc血管病变细胞和分子方面的现有知识，以及用于改善血管状况的既定和正在开发的治疗方法。
