肺泡蛋白沉积症7例临床分析

Clinical analysis of 7 patients with pulmonary alveolar proteinosis

目的探讨肺泡蛋白沉积症(PAP)的临床特点、影像学表现。方法回顾性分析7例确诊PAP患者的临床资料。结果男4例,女3例,平均年龄(51.0±6.9)岁;常见临床症状为咳嗽和进行性呼吸困难;部分患者有接触金属粉尘、特殊化学物质史;肺功能示限制性通气功能障碍5例、弥散功能障碍4例,最大通气量偏低1例。胸部CT可表现为"地图"样、"铺路石"样或间质纤维化样改变;4例经支气管镜肺活检(TBLB)和支气管肺泡灌洗(BALF),3例经胸腔镜肺活检确诊。结论 PAP患者病程长,咳嗽、活动后气促常见;肺部影像学有较特征性改变,BALF和(或)TBLB是确诊PAP的重要手段。

Objective To analyze the clinical and radiological characteristics of pulmonary alveolar proteinosis（PAP）.Methods Clinical data of 7 patients with confirmed PAP were analyzed retrospectively.Results Seven patients（4 male and 3 female） aged（51.0±6.9） years old.PAP presented a protracted chronic clinical course with few physical signs.Dyspnea after exertion and cough were the main complains.Some patients had a history of metal dusts and special chemical substance exposure.The pulmonary function tests showed restrictive ventilatory disorder in 5 cases and decreased lung diffusing capacity in 4 cases,and decreased maximal voluntary ventilarion in 1 case.Chest plain radiography showed diffuse airspace opacities,which usually located in the lower lobes and was similar to the signs of cardiogenic pulmonary edema.Characteristic ＂geographic＂,＂crazy paving＂ or interstitial fibrosis-like changes were seen on chest CT scan.Four cases were diagnosed by rideo-assisted transbronchoscopic lung biopsy（TBLB） and brochoalveolar lavage fluid（BALF） with periodic acid-schiff staining and electromicroscopic examination,3 cases by thoracscopy lung biopsy.Conclusion The patients with PAP often have cough and long-term dyspnea after exertion.There are characteristic changes on chest CT.TBLB and BALF are the important diagnostic tools for the diagnosis of PAP.