Guo Bingpeng, Wang Lulin, Xia Shu, Mao Mengmeng, Qian Weiping, Peng Xiaomin, Zheng Zexuan, Chen Rongchang, Han Qian, Luo Qun
State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Shenzhen Institute of Respiratory Diseases, Shenzhen People's Hospital, Shenzhen, China.
J Thorac Dis. 2020 Jul;12(7):3688-3696. doi: 10.21037/jtd-19-4021.
Reported data on the disease spectrum of interstitial lung diseases (ILDs) of China are sparse and varied. We aimed to investigate the spectrum of ILDs and the distribution of diagnostic methods under a uniform diagnosis.
This retrospective study enrolled ILDs cases from Guangzhou Institute of Respiratory Health (GIRH). All cases were classified into specific subgroups of ILDs according to updated guidelines.
A total of 1,945 subjects were enrolled from January 2012 to December 2017. The mean age was 57.9 years, and 1,080 (55.5%) patients were male. The most common subtype of ILDs was idiopathic pulmonary fibrosis (IPF, 20.3%), followed by interstitial pneumonia with autoimmune features (IPAF, 17.9%), connective tissue disease associated ILD (CTD-ILD, 18.3%) and unclassifiable idiopathic interstitial pneumonia (UIIP, 14.7%). A total of 818 (42.1%) patients underwent lung biopsy in order to obtain a histological diagnose. TBLB was performed in 565 (29.0%) patients, eleven of whom underwent SLB because TBLB failed to obtain lung samples. SLB was performed in 213 (11.0%) patients and TBCB was performed in 51 (2.6%) patients. Among them, histological results were considered clinically helpful in 722 (88.3%) cases, and provided definitive histopathological diagnoses in 368 cases. Surgical lung biopsy (SLB) was performed in 213 (10.9%) subjects, while 115 (54.0%) cases were performed among the idiopathic interstitial pneumonia (IIP). Among SLB cases in IIP, the highest rate of SLB was desquamative interstitial pneumonia/respiratory bronchiolitis-interstitial lung disease (DIP/RB-ILD, 10/10), lymphoid interstitial pneumonia (LIP, 9/9), followed by cryptogenic organizing (COP, 18/26), nonspecific interstitial pneumonia (NSIP, 22/53), IPF (43/395), UIIP (13/285).
IPF was the most common ILDs in our ILD center, followed by IPAF, CTD-ILD and UIIP. Histological information may help to establish diagnostic algorithm in ILD.
关于中国间质性肺疾病（ILDs）疾病谱的报道数据稀少且各不相同。我们旨在调查ILDs的疾病谱以及统一诊断下诊断方法的分布情况。
这项回顾性研究纳入了广州医科大学附属第一医院国家呼吸系统疾病临床医学研究中心（GIRH）的ILDs病例。所有病例均根据最新指南分为ILDs的特定亚组。
2012年1月至2017年12月共纳入1945名受试者。平均年龄为57.9岁，男性1080例（55.5%）。ILDs最常见的亚型是特发性肺纤维化（IPF，20.3%），其次是具有自身免疫特征的间质性肺炎（IPAF，17.9%）、结缔组织病相关ILD（CTD-ILD，18.3%）和无法分类的特发性间质性肺炎（UIIP，14.7%）。共有818例（42.1%）患者接受了肺活检以获得组织学诊断。565例（29.0%）患者进行了经支气管肺活检（TBLB），其中11例因TBLB未能获取肺组织样本而接受了外科肺活检（SLB）。213例（11.0%）患者进行了SLB，51例（2.6%）患者进行了经胸壁肺活检（TBCB）。其中，722例（88.3%）病例的组织学结果被认为对临床有帮助，368例提供了明确的组织病理学诊断。213例（10.9%）受试者进行了外科肺活检（SLB），其中115例（54.0%）在特发性间质性肺炎（IIP）中进行。在IIP的SLB病例中，SLB发生率最高的是脱屑性间质性肺炎/呼吸性细支气管炎-间质性肺疾病（DIP/RB-ILD，10/10）、淋巴细胞间质性肺炎（LIP，9/9），其次是隐源性机化性肺炎（COP，18/26）、非特异性间质性肺炎（NSIP，22/53）、IPF（43/395）、UIIP（13/285）。
IPF是我们ILD中心最常见的ILDs，其次是IPAF、CTD-ILD和UIIP。组织学信息可能有助于建立ILD的诊断算法。
