周惠琼;张奉春;
ZHOU Hui-qiong;ZHANG Feng-chun;Department of Rheumatology,The First Affiliated Hospital of Chinese PLA General Hospital;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:解放军总医院第一附属医院风湿科
• 2: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
• 3: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)：

间质性肺疾病(interstitial lung disease,ILD)是以弥漫性肺实质、肺泡炎症和间质纤维化为基本病理表现的慢性进展型疾病,缺乏特异性诊断方法。血清学标志物,特别是涎液化糖链抗原-6(Krebs von den Lungen-6,KL-6),正日益受到人们关注。KL-6是一种跨膜高分子量黏蛋白,主要表达于Ⅱ型肺泡细胞和支气管上皮细胞的细胞质和胞膜。近年来,大量研究发现KL-6表达可能与肿瘤、部分新生儿肺部疾病、ILD及其恶化、急性肺损伤等疾病相关,其在ILD中的作用受到特别关注,但具体机制仍不清楚。本文对KL-6的结构特点,其在ILD患者血清及肺泡灌洗液中的异常表现与ILD病变发生、进展、药物疗效及预后的相关性作一综述,以期为临床研究提供最新信息。
Interstitial lung diseases( ILDs) are a group of diverse pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. There are no specific diagnostic methods for ILDs currently and various serum biomarkers,including Krebs von den Lungen-6( KL-6),have been tested for their use in diagnosing ILDs. However,the pathophysiological role of KL-6 in patients with ILDs is not yet very clear. KL-6 is high-molecular-weight glycoprotein and moderately expressed in type Ⅱ pneumocytes and respiratory bronchiolar epithelial cells. KL-6 has been found to have an association with various types of diseases such as cancers,some neonatal lung diseases,ILDs,and acute lung injury. In this article,we review the structure of KL-6 and the relationship between the elevation of KL-6 and the etiology,progression,treatment evaluation,and prognosis of ILDs.

关键词(KeyWords)： 涎液化糖链抗原-6;间质性肺疾病;血清学标志物
Krebs von den Lungen-6;interstitial lung disease;serum biomarker

Abstract:

Keywords:

基金项目(Foundation):

作者(Authors): 周惠琼;张奉春;
ZHOU Hui-qiong;ZHANG Feng-chun;Department of Rheumatology,The First Affiliated Hospital of Chinese PLA General Hospital;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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