肺泡蛋白沉着症的临床和影像学分析

Clinical and Imageologic Analysis of Pulmonary Alveolar Proteinosis

目的 :探讨肺泡蛋白沉着症(PAP)的临床、影像特点以及诊断。材料和方法 :对15例经纤维支气管镜活检或开胸活检病理及肺泡灌洗证实的原发性肺泡蛋白沉着症患者的胸片和CT表现进行回顾性分析。并比较全肺灌洗治疗前、后胸部影像与动脉血气、肺功能及临床表现间的关系。结果 :PAP胸部影像表现呈多样化 ,可归纳为 :地图样表现、碎石路样表现、肺实变表现、肺水肿样表现及肺间质纤维化样表现等。但肺部阴影相对稳定(即短期内如无感染 ,胸部阴影不会发生显著变化) ,胸部影像与临床症状常不平衡。结论 :肺部影像可以充分显示肺泡蛋白沉着症的特点 ,也是随访观察临床疗效和预后转归的最重要指标之一。临床和影像相结合可以有效确诊本病。

Purpose:To study the clinical,imageologic features and diagnosis of pulmonary alveolar proteinosis(PAP).Materials and Methods:The CT and chest film findings of 15 PAP pateints(age ranging 28 to 60 years,confirmed with histopathologic and bronchial alveolar lavage) were retrospectively analyzed,and compared the relationship of images,pulmonary test and clinical symptoms before and after bronchial alveolar lavage.Results:The radiologic features of PAP could be characterized as geographic,the 'crazy-paving' pattern,lobar or segmental consolidation and like pulmonary edema or intersititial fibrosis.The manifestations were stabile and more serious than the symptoms.Conclusion:The features on chest CT and X-ray film can reveal the characteristics of PAP,and assess curative effect and prognosis of PAP.Combination of clinical symptoms and imageologic features could be drawn to the diagnosis of PAP.