肺泡蛋白沉积症诊治分析

Diagnosis and Treatment of Pulmonary Alveolar Proteinosis

目的通过临床病例分析,了解肺泡蛋白沉积症的临床和影像学表现,以提高诊断水平。方法回顾性分析确诊的6例PAP患者的临床资料。结果该病以20～50岁多见,病程慢性迁延,主要症状活动后气促,咳嗽等,特征性体征较少,部分患者有接触金属粉尘特殊化学物质史,胸部X线以两肺下叶片状模糊影为主,磨玻璃影及实变影;类似心源性肺水肿,HRCT可见"地图样"改变或"铺路石"征的特征性表现,6例支气管肺泡灌洗及病理分析。结论两肺弥漫性病变,X线类似心源性肺水肿,胸部HRCT病灶呈地图样,碎石片样;尤其中年男性,病程长,活动后气促,肺部体征相对较少,应警惕本病可能,尽早作TBLB/BALF,必要时胸腔镜肺活检确诊,提高诊断准确率。

Objective To evaluate the clinical and radiological manifestation of pulmonary alveolar proteinosis and improve the diagnosis ability of this rare disease through clinical cases analysis. Methods Retrospectively analyzing 6 the clinical characteristics of patients with confirmed pulmonary alveolar proteinosis（PAP） involved from 2003 until now. Results There were 4 male and 2 female patients with the average age of 47 （35 - 59） years old. PAP presented aprotracted chronic clinical course but few physicalsigns. The complains included dyspnea ofter exertion and cough. Some of them had exposed to metal dusts and special chemical substance. Chest plain radiography showed diffuse air space opacities, which usually located in the lower lobes and was similar to cardiogenic pulmonary edema. Characteristic chest CT showed＂geo- graphic＇or＂crazy paving＇changes. Six cases were diagnosed by rideo-assisted brochoalveolar lavage fluid（BALF）. Conclusion It should be alert to PAP when the following happens：patient with bilateral diffuse air space opacities,mostly located in the lower lobes and was similar to cardiogenic pulmonary edema in chest X-ray ; ＂geographic＂ or＂ crazy paving ＂changes in chest CT; middle age patients, especially male with long-term dyspnea ofter exertion and without obvious lung physical sign. It is necessary to under take the examination of TBLB,BALF,and thoracscopy lung biopsy for getting the accurate diagnosis.