作者：刘玲，高宝安，鲁明骞，方芳，马潇枭

单位：

1.443003湖北省宜昌市，三峡大学第一临床医学院 宜昌市中心人民医院呼吸与危重症医学科　 2.430000湖北省武汉市第三医院呼吸与危重症医学科　 3.443003湖北省宜昌市，三峡大学第一临床医学院 宜昌市中心人民医院肿瘤科 通信作者：鲁明骞，E-mail：lumingqian001@163.com

Units：

1.Department of Respiratory and Critical Care Medicine, the First College of Clinical Medical Science, China Three Gorges University/Yichang Central People's Hospital, Yichang 443003, China 2.Department of Respiratory and Critical Care Medicine, Wuhan Third Hospital, Wuhan 430000, China 3.Department of Oncology, the First College of Clinical Medical Science, China Three Gorges University/Yichang Central People's Hospital, Yichang 443003, China Corresponding author: LU Mingqian, E-mail: lumingqian001@163.com

关键词：

肺泡蛋白沉积症; 全肺灌洗术; 粒细胞-巨细胞集落刺激因子; 病例报告;

Keywords：

Pulmonary alveolar proteinosis; Whole lung lavage; Granulocyte-macrophage colony-stimulating factor; Case reports

CLC：

DOI：

10.12114/j.issn.1008-5971.2022.00.274

Funds：

吴阶平医学基金会临床科研专项资助基金（320.6750.19094-55）

摘要：

肺泡蛋白沉积症是一种罕见的肺部弥散性疾病，其特征是肺泡腔及终末呼吸性细支气管内肺泡表面活性物质异常沉积，临床上多表现为进行性加重的劳力性呼吸困难、咳嗽和低氧血症，缺乏特异性，容易出现漏诊、误诊和误治。全肺灌洗术是其经典一线疗法，目的是清除蓄积的肺泡表面活性物质，但部分患者仅行全肺灌洗术，治疗效果不佳。本文报道1例经全肺灌洗术联合重组人粒细胞-巨细胞集落刺激因子雾化吸入治疗的自身免疫性肺泡蛋白沉积症患者，并复习相关文献总结其临床表现、影像学改变、病理特征及治疗、预后，以提高临床医师对该病的诊疗水平。

Abstract：

【Abstract】 Pulmonary alveolar proteinosis (PAP) is a rare diffuse disease of the lung, characterized by the accumulation of pulmonary surfactant in alveolar space and terminal respiratory bronchioles. Clinical manifestations of PAP include progressive aggravating exertional dyspnea, cough and hypoxemia, which is easy to be missed diagnosis, misdiagnosis and mistreatment for its lack of specificity. Whole lung lavage (WLL) is classically the first-line treatment for PAP, its aim is to remove the accumulated pulmonary surfactant. However, some patients had poor therapeutic effect with WLL alone. This paper reports a case of autoimmune PAP treated by WLL combined with aerosol inhalation of recombinant human granulocyte-macrophage colony stimulating factor, and review the relevant literature and sunmmarize the clinical manifestations, imaging changes, pathological features, treatment and prognosis of PAP in order to improve the level of diagnosis and treatment of the disease of clinicians.

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