肺泡蛋白沉积症1例报告并文献复习

Report of a case of pulmonary alveolar proteinosis with literature review

目的探讨肺泡蛋白沉积症(PAP)的临床特征及诊断。方法对1例患者的临床资料进行分析并复习相关文献。结果PAP发病呈特发性或继发于其他疾病,病程慢性迁延,以进行性呼吸困难为主要表现,CT呈双肺毛玻璃样改变。支气管肺泡灌洗液呈"牛奶样"改变且提示细支气管及肺泡腔内充满过碘酸雪夫(PAS)阳性的无细胞颗粒状物质。结论PAP临床表现不具有特异性,影像学检查及诊断性支气管肺泡灌洗有助于诊断,局部或全肺灌洗疗效肯定。

Objective To discuss the clinical manifestations and diagnosis of a patient with pulmonary alveolar proteinosis （PAP）. Methods One case of PAP was analyzed and the recent literature was reviewed. Results PAP presented a protracted chronic clinical course , It was manifested as primary or secondary pathological changes. PAP was characterized by progressive dyspnea. Typical CT scan showed groundglass opacities and “milky” effluent from bronchoalveolar lavage fluid （BALF）.The microscopic characteristics of BALF from bronchoalveolar lavage of PAP indicated the alveolar space and terminal bronchioles almost completely filled with periodic Acid Schiff （PAS）--positive surfactant. Conclusion The manifestations of PAP is not specific, image examinations and diagnostic bronchoalveolar lavege are very useful and lung lavage especially whole lung lavage is effective.