结缔组织病肺间质病变的临床、影像和病理诊断

目的了解结缔组织病肺间质病变（connective tissue disease-interstitial lung disease,CTD-ILD）的临床、影像及病理学特征,探讨CTD-ILD影像学与病理类型间的相关性。方法通过高分辨计算机体层成像（high-resulution computed tomography,HRCT）技术了解132例弥漫性结缔组织病合并肺间质病变患者的肺间质病变影像学特点及分型,经皮肺穿刺活检技术进行肺间质病变病理分型。结果与系统性红斑狼疮（systemic lupuserythematosus,SLE）、多发性肌炎（polymyositis,PM）、系统性硬化症（systemic sclerosis,SSc）、类风湿关节炎（rheumatoid arthritis,RA）相比,原发性干燥综合征（primary Sj觟gren＇s syndrome,pSS）、皮肌炎（dermatomysitis,DM）、血管炎肺间质病变的呼吸道症状更明显。从影像学上看CTD-ILD表现多种多样：SLE以磨玻璃影多见,pSS以实变影多见,DM、PM、血管炎以实变影及毛玻璃影多见,SSc和RA以网格影多见。不同疾病肺间质病理类型有显著性差异,SLE、pSS、DM以非特异性肺间质炎（nonspecific interstitial pneumonia,NSIP）为主,SSc以寻常型肺间质炎（usual interstitial pneumonia,UIP）为主。病理类型NISP在影像学上多表现为毛玻璃样实变病变,UIP则更多表现为网格样蜂窝样病变,与其他影像学表现比较有统计学差异（P〈0.05）。用力肺活量和一氧化碳弥散能力下降在病种、影像学及病理改变上均无统计学差异。结论HRCT对诊断CTD-ILD有高敏感性及特异性,影像学改变可帮助临床医生推测其组织病理类型,便于随访和疗效的评价,减少肺活检的危险,是目前诊断CTD-ILD的重要手段。

Clinic,Imaging, and Histopathological Diagnosis in Connective Tissue Disease Related Interstitial Lung Disease

Objective To understand the clinical, imaging, and pathological features of connective tissue diseases with interstitial lung disease （CTD-ILD）. To investigate the relationship between imaging and pathological types. Methods 132 cases with CTD-ILD have been selected. High-resolution computed tomography （HRCT） was used to analyze the imaging features and sub-type of the CTD-ILD. Pathological types were obtained by percutaneous lung biopsy. Results Compared with SLE, PM, SSc, amd RA, the respiratory symptoms of ILD of pSS, DM, and vasculitis were more serious. Varied imaging features were shown in CTD-ILD, commonly ground-glass opacity in SLE, consol in pSS, consol and ground-glass opacity in DM/PM/vasculitis, reticulation in SSc and RA. Among different diseases, the pathological types of the lung were significantly different, the main pathological type in SLE, pSS, DM was nonspecific interstitial pneumonia （NSIP）, but usual interstitial pneumonia （UIP） dominated in SSc. The relationship between pathological types and imaging was that NISP often corresponds to ground-glass opacity and UIP usually corresponds to reticulation, which was statistically different compared to other imaging features （P 〈 0.05）. There was no difference in decline of both forced vital capacity （FVC） and diffusing capacity of the lung for carbon monoxide （DLCO） in entity, imaging or pathology. Conclusion HRCT has high sensitivity and specificity in diagnosis of CTD-ILD, the image change can help doctors to infer the histopathological types, and help to reduce the risk of lung biopsy. HRCT is the most important means in the diagnosis of CTD-ILD.