探讨肺泡蛋白沉积症的影像学意义

Explore the imaging findings of pulmonary alveolar proteinosis

目的探讨肺泡蛋白沉积症（PAP）的胸片及CT影像学表现特点及诊断价值。方法回顾性分析经病理及肺泡灌洗证实的11例PAP的临床和X线胸片、CT表现。结果PAP的主要临床症状为进行性呼吸困难、气促、咳嗽。胸片表现为两肺对称或不对称分布的斑点状、务索状、小片或大片状实变影。CT上表现多样化，可见毛玻璃征、碎石路征、地图征、支气管充气征、蜂房征、局限性肺气肿等。结论PAP是一种罕见的弥漫性肺实质病变，胸片表现缺乏特征；毛玻璃征、碎石路征、地图征等支气管充气征为CT特征表现，能对正确诊断提供重要价值。

Objective To explore the chest X- ray and CT findings and the diagnostic value of pulmonary alveolar proteinosis （PAP）. Methods Eleven cases proven by pathology or bronchoalveolar lavage were retrospectively analyzed. Results The chief clinical manifestations was progressive dyspnea, breathlessness and cough. The chest radiography presented symmetrical or asymmetrical diffuse mottling, striped, and small or large patches of ground - glass opacity. But on CT, there were diffuse patchy shadows and ground glass patterns, crazy -pavins sign, map sign, air- brochogram sign, honeycomb sign, and local emphysema. Conclusions Pulmonary alveolar proteinosis is a kind of exceptional diffuse lung parenchyma disease. The chest radiography has been lack of characteristic imaging,but CT has characteristics of PAP such as geographical patterns, group - glass patterns crazy - paving sign, honeycomb sign, air - brochogram sign,and is more valuable for accurate diagnosis of PAP.