肺泡蛋白沉积症的临床特征  

Clinical characteristics of pulmonary alveolar proteinosis

作　　者：龙颖姣 刘贵钱 彭红[1] 陈燕[1] 陈平[1] 欧阳若芸[1] LONG Yingjiao;LIU Guiqian;PENG Hong;CHEN Yan;CHEN Ping;OUYANG Ruoyun(Department of Respiratory and Critical Care Medicine,Second Xiangya Hospital/Research Unit of Respiratory Disease,Central South University/Respiratory Disease Diagnosis and Treatment Center of Hunan Province,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院呼吸与危重症医学科,中南大学呼吸疾病研究所,湖南省呼吸疾病诊疗中心,长沙410011

出　　处：《中南大学学报（医学版）》2021年第2期156-161,共6页Journal of Central South University ：Medical Science

基　　金：国家重点临床专科建设项目(2012-650);国家自然科学基金(81800043);国家重点研发计划(2016YFC0901502)。

摘　　要：目的:肺泡蛋白沉积症(pulmonary alveolar proteinosis,PAP)是一种少见的肺弥漫性疾病,其临床表现无特异性且差异很大,临床医生对该病的诊断和治疗认识尚不足,易造成误诊。本研究通过分析PAP的临床表现、影像学特点、诊断及治疗方法,以期提高对该疾病的诊疗水平。方法:回顾性分析2008至2019年中南大学湘雅二医院呼吸与危重症医学科病理确诊的25例PAP患者的临床资料。结果:不明原因的咳嗽以及气促为PAP常见的临床症状;5例患者有职业吸入暴露史;16例患者具有典型胸部影像学表现,影像学特征包括多发磨玻璃样病变,呈地图样分布,铺路石征以及弥漫性网格状影;14例接受肺功能检测,主要表现为弥散功能受损;经支气管镜肺活检病理诊断PAP阳性率为95%。5例行全肺灌洗术,5年后随访患者症状和胸部影像学表现改善。结论:PAP有特征性影像学及病理表现。经支气管镜肺活检病理诊断是确诊PAP的主要方式,全肺灌洗术是PAP经典且有效的治疗方法。Objective:Pulmonary alveolar proteinosis(PAP)is a rare disease with non-specific and various clinical manifestations,often leading to misdiagnosis.This study aims to raise the awareness of this disease via summarizing the clinical characteristics,diagnosis,and therapy of PAP.Methods:We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.Results:Cough with unkown reason and dyspnea were common clinical manifastations of PAP.Five patients had a history of occupational inhalational exposure.Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa,in typical shapes called crazy-paving and geographic pattern.Fourteen patients underwent pulmonary function tests,revealing a reduction in the diffusing capacity for carbon monoxide.The positive rate of transbronchial biopsy was 95%.Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.Conclusion:PAP is characterized by radiographic pattern and pathology.Transbronchial lung biopsy is effective to make diagnosis of PAP.The whole lung lavage remains a efficient therapy.

关 键 词：肺泡蛋白沉积症 临床特征 影像学表现 

分 类 号：R563.9[医药卫生—呼吸系统]