摘要：目的 通过临床分析及文献复习,探讨肺泡蛋白沉着症(PAP)的临床表现、诊断方法 、治疗及预后,提高对该病的认识.方法 回顾性分析12例PAP患者的临床资料.结果 活动后气促和咳嗽是PAP的主要症状,体征较少,肺部影像学表现多样化,肺功能主要为限制性通气功能障碍和弥散功能障碍,需病理确诊.结论 PAP临床体征与影像学表现不平衡,影像学表现较体征重,易误诊、漏诊.加强对该病的认识是提高诊断率的关键. Abstract： Objective To improve the understanding of pulmonary alveolar proteinosis (PAP) by exploring clinical manifestation, diagnosis,treatment and prognosis of PAP through clinical analysis and the literature review. Methods Retrospectively analysis of clinical characteristics of 12 patients with PAP.Results PAP,with few physical signs, whose common symptoms included dyspnea after physical activities and cough. The radiological features of PAP could be characterized as geographic.The pulmonary function of PAP appeared restriction of ventilation function and impairment of lung diffusion function. The accurate diagnosis of PAP need pathologic evidence. Conclusions The typical features are stabilization of the shadow and that the shadow is more serious than the symptoms, often causing a significant delay in the diagnosis or misdiagnosis.Increase the knowledge of PAP is crucial in improving the rate of diagnosis.

关键词：肺泡蛋白沉着症 pap 影像学表现 pulmonary alveolar 

单位：郑州大学第一附属医院呼吸科 450052 河南省人民医院呼吸科