肺泡蛋白沉积症15例临床分析

目的 分析肺泡蛋白沉积症（PAP）的临床表现、辅助检查、诊断方法及治疗效果,以提高临床医生对该疾病的认识。方法 回顾性分析2005～2019 年15 例PAP 住院患者的临床资料（包括年龄、性别、职业暴露、临床症状及体征、辅助检查、治疗方法等）。结果 患者临床表现主要为咳嗽、呼吸困难、胸闷,肺功能提示弥散功能下降,影像学检查主要表现为双肺间质性改变,典型特征为“铺路石征”“地图征”改变。确诊主要依靠肺活检或肺泡灌洗液病理明确。9 例患者进行全肺灌洗术治疗,1 例患者接受雾化吸入治疗。治疗后临床症状均有缓解。结论PAP 是一种少见的间质性肺病,由于其缺乏特异性的临床表现,在诊断PAP 时常常出现误诊漏诊。熟悉掌握PAP的临床表现及影像学特征,可以减少PAP 的误诊漏诊,更好地服务临床。

Clinical analysis of 15 cases of pulmonary alveolar proteinosis

Objective To analyze the clinical manifestations,auxiliary examinations,diagnostic methods and therapeutic efficacies of pulmonary alveolar proteinosis(PAP),so as to improve clinicians'' understanding of PAP.Methods The clinical data of 15 cases of PAP inpatients from 2005 to 2019 were analyzed retrospectively,in which the age,gender,occupational exposure,clinical symptoms,physical signs,auxiliary examination,and treatment methods,etc.were included.Results The main clinical manifestations were cough,dyspnea,chest distress,and pulmonary function suggested decreased diffusion function.Imaging examination mainly showed interstitial changes of both lungs,with typical features of cobble-stone sign and map-like changes.The diagnosis was mainly confirmed by pulmonary biopsy or bronchoalveolar lavage fluid pathology.9 patients were treated with whole lung lavage,and 1 patient was treated with atomizing inhalation.Clinical symptoms were relieved after treatment.Conclusion PAP is a rare interstitial lung disease.Because of its lack of specific clinical manifestations,it is often misdiagnosed and missed in the diagnosis of PAP.Being familiar with the clinical manifestations and imaging features of PAP can reduce misdiagnosis and missed diagnosis of PAP and better serve the clinic.