目的：分析弥漫性间质性肺疾病（ diffuse interstitial lung disease， DILD，简称ILD）的疾病谱分布情况及临床特征，以提高诊疗水平。方法收集111例2006～2013年在北京协和医院住院的弥漫性肺间质性疾病患者的临床资料，回顾性分析其病因、临床表现、肺功能、血气分析及病理特征等。结果患者以咳嗽、呼吸困难为主要临床表现，近一半患者存在低氧血症。弥漫性间质性肺疾病与继发性结缔组织疾病相关性弥漫性肺间质疾病（ CTD－ILD）相比杵状指的发生率较高，而后者有特殊接触史者较多（ P＜0.05）。83．9％的患者存在限制性通气功能障碍。高分辨率计算机断层扫描影像主要表现为斑片、淋巴结肿大、毛玻璃样改变、胸膜病变、结节和网格影。特发性间质肺炎（IIP）与继发性ILD相比，后者结节影发生率更高（P＜0．05）。 ILD主要病理类型为非特异性间质肺炎（NSIP）（19．8％）、外源性过敏性肺泡炎（18．9％）、CTD －ILD （11.7％）、结节病（8．1％）。结论明确不同弥漫性肺间质疾病的临床特征可减少漏诊、误诊及误治，提高诊疗水平。%Objective This study aimed to analyze the disease spectrum distribution of diffuse interstitial lung disease and increase diagnostic and therapeutic level of this kind of disease.Methods Clinical materials of one hundred and eleven patients with diffuse interstitial lung disease hospitalized into Peking Union Medical College Hospital from 2006 to 2013 were collected.Causes, symptoms, lung function, blood gas and pathological characters were analyzed retrospectively.Results Cough, dyspnea were the major symptoms and nearly half of the patients had hypoxemia.The incidence of acropachia of patients with interstitial lung disease was higher than that of the patients with interstitial lung disease associated with connective tissue disease, but the latter had more specific contact -type ( both P <0.05).83.9% patients had restrictive ventilatory functional disturbance.The major radiological characters by high-resolution computed tomography included patch, swollen lymph nodes, frosted glass like change, pleural disease, nodular and grid shadow.The incidence of nodular shadow of the patients with interstitial lung disease associated with connective tissue disease was higher than that of the patients with interstitial lung disease ( P <0.05 ) .The major pathological type of interstitial lung disease included nonspecific interstitial pneumonia (19.8%), extrinsic allergic alveolitis (18.9%), interstitial lung disease associated with connective tissue disease (11.7%), sarcoidosis (8.1%).Conclusion Confirming clinical character of diffuse lung disease could reduce missed diagnosis, misdiagnosis and mistherapy and raise diagnosis and therapeutic levels.
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