肺泡蛋白沉积症2例及文献复习

Two cases of pulmonary alveolar proteinosis with literature review

目的分析总结肺泡蛋白沉积症（PAP）的临床特征、发病机制及诊治方法。方法对2例患者的临床资料进行回顾性分析并复习相关文献。结果2例患者病程均2年以上，临床特征表现为症状、体征与影像学分离现象，行肺泡灌洗液检查及经支气管肺活检病理明确诊断，行全肺灌洗治疗后，血气分析及胸部CT有明显改善。结论PAP是一种少见的肺部疾患，临床表现不典型，但随着经支气管肺活检技术的提高及肺泡灌洗液分析的进步，被发现的病例有增多趋势，其胸部CT主要征象为地图样、铺路石样及毛玻璃样改变。病理特点是肺泡腔内充满了微小颗粒状的PAS染色阳性的物质。发病机制复杂，全肺灌洗是目前最有效的治疗方法。

Objective To summarize and analyze the clinical manifestation, pathogenesis, and treatment of pulmonary alveolar proteinosis （PAP）. Methods The clinical data of two patients were retrospectively analyzed and the related literatures were reviewed. Results The duration of disease of two patients was more than two years, the clinical manifestation of PAP was characterized by the incompatibility between symptoms signs and radiographs. They were diagnosed by alveolar lavage and transbronchoscopic lung biopsy. After whole hmg lavage therapy, blood gas and chest CT scan improved significantly. Conclusions PAP is a rare lung disease, clinical manifestation is not typical. But with the progress of transbronchoscopic lung biopsy technique and alveolar lavage fluid analysis, the found cases had a trend of increasing. Typical chest CT scan showed map, pave stone, and ground glass change. Pathological characteristics is alveolar cavity filling with small granular PAS positive substance. Its pathogenesis is complex, whole lung lavage is the most efficient therapy for PAP at present.