肺泡蛋白沉积症的诊断要点及10例临床分析

Diagnostic main point of pulmonary alveolar proteinosis: analysis of 10 cases

目的通过临床病例分析,了解肺泡蛋白沉积症(PAP)的临床和影像学表现,提高诊断水平.方法回顾分析我院自1991年来确诊的10例PAP患者的临床资料.结果①男8例,女2例,平均年龄49.7岁,以40～50岁多见.②病程慢性迁延,主要症状活动后气促,咳嗽等,体征较少.③部分患者有接触金属粉尘、特殊化学物质史.④胸部X线以两肺下叶片状模糊影为主,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.⑤5例经气管镜肺活检(TBLB)和支气管肺泡灌洗(BALF),1例经肺泡灌洗液PAS染色及病理分析,4例经胸腔镜肺活检明确.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 analysing 10 the clinical characteristics of patients with confirmed pulmonary alveolar proteinosis(PAP) involved from 1991 until now.Results ①There were 8 male and 2 female patients with the average age of 49.7 (40～50) years old.②PAP presented a protracted chronic clinical course but few physical signs.The complains included dyspnea ofter exertion and cough.③Some of them had exposed to metal dusts and special chemical substance.④Chest plain radiography showed diffuse airspace opacities,which usually located in the lower lobes and was similar to cardiogenic pulmonary edema.Characteristic chest CT showed “geographic” or “crazy paving” changes.⑤Five cases were diagnosed by rideo-assisted transbronchoscopic lung biopsy(TBLB) and brochoalveolar lavage fluid (BALF),1 case by BALF with periodic acid-schiff staining and electromicroscopic examination,4 cases by thoracscopy lung biopsy.Conclusions It should be alert to PAP when the following happens: patient with bilateral diffuse airspace opacities,mostly located in the lower lobes,and is 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 undertake the examination of TBLB,BALF,and thoracscopy lung biopsy for getting the accurate diagnosis.