陈志磊;张奉春;
CHEN Zhi-lei;ZHANG Feng-chun;Department of Rheumatology and Clinical Immunology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;The Ministry of Education Key Laboratory,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

• 1:中国医学科学院北京协和医学院北京协和医院风湿免疫科
• 2:中国医学科学院北京协和医学院北京协和医院教育部重点实验室

摘要(Abstract)：

结缔组织病(connective tissue disease,CTD)是以自身免疫及其介导的器官系统损伤为特征的一组全身性疾病。肺部并发症是CTD的重要关节外表现,也是CTD患者死亡的主要原因。临床管理CTD相关间质性肺疾病(CTD-associated interstitial lung disease,CTD-ILD)的关键是确定ILD类型、疾病所处阶段、应给予何种治疗。目前CTD-ILD仍采用特发性间质性肺炎分类,其诊断治疗尚无统一的临床指导策略。越来越多的研究发现,自身抗体与CTD-ILD相关,对临床诊治具有一定指导意义,临床实践中应更多关注那些肺部表现不明显的CTD患者,以尽早识别CTD-ILD,给予适当治疗,延缓疾病进展。
Connective tissue disease( CTD) is a group of systemic disorder characterized by autoimmunity and autoimmune-mediated organ and system damage. The pulmonary complication is an important extra-articular feature of CTD and a major cause of mortality. The key point in the management of CTD-associated interstitial lung disease( CTD-ILD) should focus on the type of ILD,the stage of the disease,and what proper measures should be taken. CTD-ILD is still using the classification of idiopathic interstitial pneumonia.However,there is no authoritative clinical guidance for the diagnosis and treatment of CTD-ILD. More and more studies show that some auto-antibodies are strongly associated with CTD-ILD,which may guide clinical practice. In clinical practice,physicians should particularly pay more attention to CTD patients with insignificant pulmonary manifestations,so as to identify CTD-ILD in the early stage and intervene at the right time to delay the progression.

关键词(KeyWords)： 结缔组织病;间质性肺疾病;结缔组织病相关间质性肺疾病
connective tissue disease;interstitial lung disease;connective tissue disease-associated interstitial lung disease

Abstract:

Keywords:

基金项目(Foundation):

作者(Authors): 陈志磊;张奉春;
CHEN Zhi-lei;ZHANG Feng-chun;Department of Rheumatology and Clinical Immunology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;The Ministry of Education Key Laboratory,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

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