免疫细胞在结缔组织病相关性肺动脉高压发病机制中的作用

IF 5.7 2区 医学 Q1 IMMUNOLOGY Frontiers in Immunology Pub Date : 2024-09-18 DOI:10.3389/fimmu.2024.1464762
Zhe Li, Juan Ma, Xuejing Wang, Liquan Zhu, Yu Gan, Baoquan Dai
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引用次数: 0

摘要

结缔组织疾病相关肺动脉高压(CTD-PAH)是一种以肺动脉压力升高为特征的疾病,是结缔组织疾病的并发症之一。CTD-PAH 患者占所有 PAH 患者的 25.3%。CTD-PAH 的主要病理特征是肺动脉内膜、中膜和外膜增厚,肺血管阻力增加,自身免疫激活和炎症反应。值得注意的是,异常的免疫激活会产生自身抗体和释放细胞因子,异常的免疫细胞募集会促进炎症环境和血管重塑。因此,几乎所有形式的结缔组织疾病都与 PAH 有关。除了 PAH 的一般治疗和靶向药物治疗外,大剂量糖皮质激素联合免疫抑制剂可迅速缓解和稳定 CTD-PAH 的基本病情。有鉴于此,开发针对免疫调节失调和炎症加剧的治疗方法被认为是预防或逆转 CTD-PAH 病程进展的一种有前途的策略。本综述探讨了免疫细胞导致 CTD-PAH 发展的潜在机制,并研究了免疫抑制疗法在治疗 CTD-PAH 中的临床应用。
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The role of immune cells in the pathogenesis of connective tissue diseases-associated pulmonary arterial hypertension
Connective tissue diseases-related pulmonary arterial hypertension (CTD-PAH) is a disease characterized by an elevated pulmonary artery pressure that arises as a complication of connective tissue diseases. The number of patients with CTD-PAH accounts for 25.3% of all PAH patients. The main pathological features of CTD-PAH are thickening of intima, media and adventitia of pulmonary arterioles, increased pulmonary vascular resistance, autoimmune activation and inflammatory reaction. It is worth noting that abnormal immune activation will produce autoantibodies and release cytokines, and abnormal immune cell recruitment will promote inflammatory environment and vascular remodeling. Therefore, almost all forms of connective tissue diseases are related to PAH. In addition to general therapy and targeted drug therapy for PAH, high-dose glucocorticoid combined with immunosuppressant can quickly alleviate and stabilize the basic CTD-PAH disease. Given this, the development of therapeutic approaches targeting immune dysregulation and heightened inflammation is recognized as a promising strategy to prevent or reverse the progression of CTD-PAH. This review explores the potential mechanisms by which immune cells contribute to the development of CTD-PAH and examines the clinical application of immunosuppressive therapies in managing CTD-PAH.
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来源期刊
CiteScore
9.80
自引率
11.00%
发文量
7153
审稿时长
14 weeks
期刊介绍: Frontiers in Immunology is a leading journal in its field, publishing rigorously peer-reviewed research across basic, translational and clinical immunology. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Immunology is the official Journal of the International Union of Immunological Societies (IUIS). Encompassing the entire field of Immunology, this journal welcomes papers that investigate basic mechanisms of immune system development and function, with a particular emphasis given to the description of the clinical and immunological phenotype of human immune disorders, and on the definition of their molecular basis.
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