目的探讨肺泡蛋白沉积症(PAP)病例的临床特征及疗效,为提高该病诊疗水平和控制病情的预后提供依据。方法回顾性分析本院确诊的12例PAP患者的临床资料,对其诊治及预后进行分析。结果 12例均经纤支镜肺组织活检和肺泡灌洗液PAS染色阳性确诊,9例患者予以大容量全肺灌洗(WLL)治疗,效果较好。结论病理学检查是确诊本病的依据,咳嗽、进行性呼吸困难是其临床特征,目前治疗的首选方法仍是全肺灌洗。 Objective To investigate the clinical features and therapeutic effects of pulmonary alveolar proteinosis (PAP) in patients with pulmonary alveolar proteinosis (PAP) and provide evidence for improving the diagnosis and treatment of the disease. Methods The clinical data of 12 patients with PAP diagnosed in our hospital were analyzed retrospectively. The diagnosis, treatment and prognosis were analyzed. Results All the 12 cases were diagnosed by PBC staining of bronchofibroscopy and bronchoalveolar lavage fluid. Nine patients were treated with high volume whole lung lavage (WLL). Conclusion Pathological examination is the basis for the diagnosis of the disease, cough, progressive dyspnea is its clinical features, the preferred method of treatment is still the whole lung lavage.