肺泡蛋白沉着症10例临床分析

Clinical analysis of pulmonary alveolar proteinosis

目的通过临床病例分析,了解肺泡蛋白沉着症(PAP)的临床和影像学表现,提高诊断水平。方法回顾分析确诊的10例PAP患者的临床资料。结果男8例,女2例,平均年龄46.7岁。病程慢性迁延,主要症状活动后气促,咳嗽等,体征较少。胸部影像表现呈多样化,可归纳为:地图样表现、碎石路样表现、肺实变样表现、肺水肿样表现及肺间质纤维化样表现等。10例经支气管镜肺活检(TBLB)和支气管肺泡灌洗(BALF)明确。结论肺部影像可以充分显示肺泡蛋白沉积症的特点,尽早作TBLB、BALF确诊。

Objective To evaluate the clinical and radiological manifestation of pulmonary alveolar proteinosis and to improve the diagnosis ability of this rare disease through clinical analysis. Methods Retrospectively analysis of clinical characteristics of 10 patients with confirmed pulmonary alveolar proteinsis （PAP） was conducted. Results ① There were 8 male and 2 female patients with the average age of 46.5, ranging between 40 and 50. ② PAP presented a protracted chronic clinical course with few physical signs. The complains included dyspnea after physical activities and cough. ③The radiological features of PAP could be characterized as geographic, the ＂crazy -paving＂ pattern, lobar or segmental consolidation, pulmonary edema or interstitial fibrosis alike. ④ 10 cases were diagnosed by radio-assisted transbronchoscopic lung biopsy （TBLB） and bronchoalveolar lavage fluid （BALF）. Condusions The features on chest CT and X-ray films can reveal the characteristics of PAP. It is necessary to undertake the examinations of TBLB and of BALF to make the ac- curate diagnosis.