Juvet Stephen C, Hwang David, Waddell Thomas K, Downey Gregory P
Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Can Respir J. 2008 May-Jun;15(4):203-10. doi: 10.1155/2008/528948.
The present article is the second in a series on rare lung diseases. It focuses on pulmonary alveolar proteinosis (PAP), a disorder in which lipoproteinaceous material accumulates in the alveolar space. PAP was first described in 1958, and for many years the nature of the material accumulating in the lungs was unknown. Major insights into PAP have been made in the past decade, and these have led to the notion that PAP is an autoimmume disorder in which autoantibodies interfere with signalling through the granulocyte-macrophage colony-stimulating factor receptor, leading to macrophage and neutrophil dysfunction. This has spurred new therapeutic approaches to this disorder. The discussion of PAP will begin with a case report, then will highlight the classification of PAP and review recent insights into the pathogenesis of PAP. The approach to therapy and the prognosis of PAP will also be discussed.
本文是关于罕见肺部疾病系列文章的第二篇。它聚焦于肺泡蛋白沉积症（PAP），这是一种脂蛋白物质在肺泡腔中积聚的病症。PAP于1958年首次被描述，多年来，肺部积聚物质的性质一直不明。在过去十年中，对PAP有了重大见解，这些见解导致了这样一种观念，即PAP是一种自身免疫性疾病，其中自身抗体干扰通过粒细胞 - 巨噬细胞集落刺激因子受体的信号传导，导致巨噬细胞和中性粒细胞功能障碍。这促使了针对该疾病的新治疗方法的出现。对PAP的讨论将从一个病例报告开始，然后将突出PAP的分类并回顾对PAP发病机制的最新见解。还将讨论PAP的治疗方法和预后。
