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REVIEW article

Front. Immunol.
Sec. Inflammation
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1464762
This article is part of the Research Topic Community Series in Inflammation in Respiratory and Neurological Diseases and the immune-interaction of the lung-brain axis: Volume II View all 8 articles

The Role of Immune Cells in the Pathogenesis of Connective Tissue Diseases-Associated Pulmonary Arterial Hypertension

Provisionally accepted
Zhe Li Zhe Li 1Juan Ma Juan Ma 1Xuejing Wang Xuejing Wang 2Liquan Zhu Liquan Zhu 1Yu Gan Yu Gan 1Baoquan Dai Baoquan Dai 1*
  • 1 Weifang Maternal and Child Health Hospital, Weifang, China
  • 2 School of Rehabilitation Medicine, Shandong Second Medical University, Weifang, Shandong Province, China

The final, formatted version of the article will be published soon.

    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.

    Keywords: Inflammation, Immunity, Cytokines, Chemokines, pulmonary hypertension, immunosuppressive therapy

    Received: 15 Jul 2024; Accepted: 02 Sep 2024.

    Copyright: © 2024 Li, Ma, Wang, Zhu, Gan and Dai. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Baoquan Dai, Weifang Maternal and Child Health Hospital, Weifang, China

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.