肺泡蛋白沉积症影像学诊断

Imaging Diagnosis of Pulmonary Alveolar Proteinosis

目的：探讨肺泡蛋白沉积症的影像学诊断。材料与方法：搜集肺泡蛋白沉积症4例，其中3例由支纤镜检及肺泡灌洗术证实，1例尸检证实。均摄有胸部正侧位片，其中1例进行了CT检查。结合国内外相关文献及尸检病理所见，对其影像学表现进行回顾性分析并探讨其病理基础。结果：该病依病程长短可分别具有下列不同的影像学表现：①弥散分布的非肿瘤性腺泡结节，为较早期表现；②弥漫性斑片状阴影与磨玻璃影，CT扫描病灶常呈地图样分布；③蝶翼征，见于病程较长者；④支气管气相；⑤实变阴影或磨玻璃影衬以网状影。结论：仔细辨认X线与CT征象，明确肺部病变属腺泡型，再结合病灶形态与分布，胸内胸外有无其他改变，影像学动态对比观察及临床病史综合分析，可不依赖支纤镜或肺活检而明确诊断。

Objective: To Study the imaging diagnosis of pulmonary alveolar proteinosis (PAP). Materials and Methods: Imaging features of 4cases with PAP were analyzed retrospectively, of which 3 cases were histologically proved by fiberoptic bronchoscopy and bronchoalveolar lavage and1 case by autopsy. Chest X-ray film was performed in all cases and CT scan in one. Results:With the developing of the disease, the following ima-ging features were seen. (1) Diffuse, ill-defined acinus nodule, an early sign. (2) Diffuse patchy shadows and ground glass appearance, distibutedgeographically on CT. (3) Butterfly sign, usually seen in prolonged cases. (4) Air-brochogram sing. (5) Consolidation or groud glass shadow withreticular background, creating the typical 'crazy-paving' appearances. Conclusion: Carefully distinguishing the X-ray and CT appearances, confirm-ing of air-space lesion, observing the shape and distribution of the lesion, and taking the clinical history into consderation together, a reliable diag-nosis can be made, needn' t doing bronchoscopy or TNB.