肺泡蛋白沉积症32例临床分析

摘要

目的 探讨肺泡蛋白沉积症(PAP)的临床特征和诊疗方法,提高诊疗水平.方法 回顾性分析2008年5月~2015年5月确诊的32例肺泡蛋白沉积症患者的临床资料.结果 不明原因的咳嗽、渐进性呼吸困难为其常见的临床症状,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、肺实变样表现及肺间质纤维化样表现等.低氧血症和肺功能损害分别达到87.5%和90.6%,肺功能损害主要表现为限制性通气功能障碍.肺泡灌洗液和(或)肺组织活检过碘酸雪夫染色可确诊.大容量全肺灌洗治疗仍为目前确切有效缓解病情的治疗方法.结论 PAP虽然是少见病,但因其病理有特征性,使得影像学上有相对特征性的表现,临床医生宜熟悉该病的临床特点和影像学表现,及时明确诊断并予全肺灌洗术治疗.%Objective To explore the clinical features, diagnosis and therapy of pulmonary alveolus protei-nosis. Methods The clinical data of 32 patients with pulmonary alveolus proteinosis from May 2008 to May 2015 were reviewed. Results The common symptoms were gradual dyspnea and cough. The radio logical features of PAP could be characterized as geographic, crazy paving appearance, lobar or segmental consolidation and interstitial brass-ier like et. al. Hypoxemia and lung function damage was 87. 9% and 90. 9%. Lung functional manifestations were mainly restrictive ventilatory functional disturbance. The way to make a definite diagnosis was lung biopsy and bron-choalveolar lavage fluid PAS dyeing. The whole lung lavage was still the most efficient and safety therapy. Conclu-sion PAP has characteristic maifestations in imaging due to its pathological features though it is rare. The doctor should know its clinical features and imageology well so as to take the whole lung lavage and other related treatments timely.