147例常见结缔组织病相关间质性肺病的临床特征及转归分析

Clinical characteristics and treatment outcome of 147 cases of common connective tissue disease-related interstitial lung disease

目的探讨常见结缔组织病相关间质性肺病(CTD-ILD)的临床及影像学特征。方法回顾性分析2014年6月至2016年12月在西安交通大学第一附属医院风湿免疫科住院治疗的147例常见CTD-ILD患者的一般情况、临床表现、血气分析、肺功能检测及胸部高分辨CT等临床资料。结果147例常见CTD-ILD患者,平均年龄(56.2±12.3)岁,女性为主(72.1%)。皮肤病变(51.7%)、乏力(50.3%)最为常见,高于呼吸系统症状如气短(47.6%)、咳痰(36.0%)、咳嗽(32.0%)。近一半(49.6%)患者出现低氧血症,肺功能改变多表现为混合性通气障碍伴弥散功能障碍。肺部影像学以磨玻璃影(78.3%)为主要特征,最常见影像学分型是非特异性间质性肺炎(NSIP),占67.2%。随访时间1~3.5年,期间病死率26.2%,其中多发性肌炎/皮肌炎(PM/DM)(66.7%)高于系统性硬化症(SSc)(15.1%)、类风湿性关节炎(RA)(15.1%)及干燥综合征(SS)(3.0%)。死亡原因主要为感染14例(42.4%)及原发疾病急性进展11例(33.3%)。结论常见CTD-ILD患者好发于女性,皮肤改变、乏力等全身症状发生率高于气短、咳嗽、咳痰等呼吸系统症状,病死率较高,主要原因是感染及疾病急性进展,进行血气分析、肺功能及胸部影像学检查对疾病诊疗及判断预后有重要指导意义。

Objective To investigate the clinical and imaging features of common connective tissue disease-related interstitial lung disease (CTD-ILD). Methods We made a retrospective analysis of 147 cases of common CTD-ILD patients hospitalized in the Department of Rheumatology of The First Affiliated Hospital of Xi an Jiaotong University from June 2014 to December 2016. Their clinical data including general information,manifestations,blood gas analysis,pulmonary function test,lung high resolution computed tomography (HRCT) were analyzed. Results The average age of the 147 patients with common CTD-ILD was (56.2±12.3) years and the majority of the patients were women (72.1%). Skin lesions (51.7%) and fatigue (50.3%) were the most common clinical manifestations,which were higher than respiratory symptoms such as dyspnea (47.6%),expectotation (36.0%),and cough (32.0%). Nearly half (49.6%) of the patients had hypoxemia,and pulmonary function changes were mostly manifested as mixed ventilation dysfunction with diffuse dysfunction. Pulmonary imaging was characterized by ground-glass opacity (78.3%). The most common radiographic type was non-specific interstitial pneumonia (NSIP),accounting for 67.2%. During the follow-up period of 1-3.5 years,the mortality rate was 26.2%. PM/DM (66.7%) was higher than SSc (15.1%),RA (15.1%) and SS (3.0%). The main causes of death were infections (42.4%) and acute exacerbation of CTD-ILD (33.3%). Conclusion The common CTD-ILD is predominant in women. The incidence of systemic symptoms such as skin changes and fatigue are higher than that of respiratory symptoms such as shortness of breath,cough and sputum. The mortality rate is high and the main causes are infections and acute exacerbation of CTD-ILD. Therefore,blood gas analysis,pulmonary function test and lung HRCT examination are helpful for diagnosis and treatment of CTD-ILD.