天津医药 ›› 2024, Vol. 52 ›› Issue (7): 673-678.doi: 10.11958/20240260

• 专题研究·结缔组织病-间质性肺病/肺动脉高压(主编 魏蔚) •    下一篇

结缔组织病相关间质性肺病:从发病机制研究到“双达标”治疗策略

李梦涛(), 王迁   

  1. 中国医学科学院北京协和医院风湿免疫科,国家皮肤与免疫疾病临床医学研究中心(邮编100730)
  • 收稿日期:2024-03-06 修回日期:2024-04-17 出版日期:2024-07-15 发布日期:2024-07-11
  • 作者简介:李梦涛(1972),男,教授,主任医师,博士研究生/博士后导师,全国著名风湿免疫学专家。北京协和医院风湿免疫科主任,协和学者特聘教授。国家卫生健康突出贡献中青年专家,北京医学会风湿病学分会主任委员,中华医学会风湿病学分会副主任委员,中国医师协会风湿免疫科医师分会副会长兼肺血管/间质病学组组长,国家风湿病数据中心(CRDC)及中国风湿免疫病医联体联盟(CRCA)秘书长,国家皮肤与免疫疾病临床医学研究中心(NCRC-DID)办公室主任,风湿免疫病学教育部重点实验室主任。《Rheumatology and Immunology Research(RIR)》副主编。系统性红斑狼疮研究国家十四五重点研发计划项目首席专家,十三五重点研发计划、首都协同创新重点项目及国自然课题负责人。E-mail:mengtao.li@cstar.org.cn

Connective tissue disease-associated interstitial lung disease: from pathogenesis to "dual-target" treatment strategies

LI Mengtao(), WANG Qian   

  1. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing 100730, China
  • Received:2024-03-06 Revised:2024-04-17 Published:2024-07-15 Online:2024-07-11

摘要:

结缔组织病(CTD)是一类多器官系统受累的异质性疾病,间质性肺病(ILD)是多种CTD的严重并发症。不同CTD-ILD在发病机制、临床表现、治疗等方面存在较大异质性。基于遗传学、病理生理与免疫学、蛋白质组学、微生物组学进行相关基础及临床研究来探究CTD-ILD发病机制并提出“双达标”治疗策略。影像学技术及血清生物标志物研究的进展同时助力“双达标”治疗策略的实施,早期控制CTD活动与肺部炎症、延缓肺纤维化有利于改善CTD-ILD患者生存期与生活质量。

关键词: 结缔组织疾病, 肺疾病,间质性, 诊断, 治疗, 发病机制

Abstract:

Connective tissue diseases (CTD) are a spectrum of heterogeneous diseases with multiple organ involvements. Interstitial lung diseases (ILD) are serious complications of CTD. There is great heterogeneity in pathogenesis, clinical manifestations, and treatment of different CTD-ILD. Previous basic and clinical studies have explored the pathogenesis of CTD-ILD, and the "dual-target" treatment strategy has therefore emerged. The implementation of the "dual-target" treatment strategy helps to control CTD activity and lung inflammation in the early stage, prevent the progression of pulmonary fibrosis, and thus improve patient survival and quality of life.

Key words: connective tissue diseases, lung diseases, interstitial, diagnosis, therapy, pathogenesis

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