Li Zhe, Ma Juan, Wang Xuejing, Zhu Liquan, Gan Yu, Dai Baoquan
Department 5 of Pediatric, Weifang Maternal and Child Health Hospital, Weifang, China.
School of Rehabilitation Medicine, Shandong Second Medical University, Weifang, China.
Front Immunol. 2024 Sep 17;15:1464762. doi: 10.3389/fimmu.2024.1464762. eCollection 2024.
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.
结缔组织病相关肺动脉高压（CTD-PAH）是一种以肺动脉压力升高为特征的疾病，它是结缔组织病的并发症。CTD-PAH患者数量占所有PAH患者的25.3%。CTD-PAH的主要病理特征是肺小动脉内膜、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并释放细胞因子，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病都与PAH有关。除了针对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以迅速缓解并稳定CTD-PAH基础疾病。鉴于此，针对免疫失调和炎症加剧开发治疗方法被认为是预防或逆转CTD-PAH进展的一种有前景的策略。这篇综述探讨了免疫细胞促成CTD-PAH发展的潜在机制，并研究了免疫抑制疗法在管理CTD-PAH中的临床应用。
