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肺泡蛋白质沉积症。

Pulmonary alveolar proteinosis.

机构信息

Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.

出版信息

Can Respir J. 2012 Jul-Aug;19(4):243-5. doi: 10.1155/2012/841530.

Abstract

Pulmonary alveolar proteinosis (PAP) is a disease of alveolar accumulation of phospholipoproteinaceous material that results in gas exchange impairment leading to dyspnea and alveolar infiltrates. There are three forms of PAP: congenital, acquired and idiopathic; of which the latter two are predominant in the adult population. Previous case studies have found that the acquired form can be secondary to various autoimmune, infectious, malignant and environmental etiologies. Recent advances in the understanding of the pathophysiology of PAP demonstrate that the idiopathic form is due to antigranulocyte macrophage-colony stimulating factor antibodies. Therapeutic targets that replace granulocyte macrophage colony stimulating factor or remove these antibodies are being actively developed. The current standard of care is to perform whole lung lavage on these patients to clear the alveolar space to help improve respiratory physiology. A case of PAP is reported, followed by a literature review on the diagnosis and management of this rare condition with the aim of increasing awareness among physicians when treating patients who present with alveolar infiltrates.

摘要

肺泡蛋白沉积症(PAP)是一种肺泡内磷脂蛋白样物质蓄积的疾病,导致气体交换受损,引起呼吸困难和肺泡浸润。PAP 有三种形式:先天性、获得性和特发性;其中后两种在成年人群中更为常见。既往病例研究发现,获得性形式可能继发于各种自身免疫、感染、恶性和环境病因。对 PAP 病理生理学的最新认识表明,特发性形式是由于抗粒细胞-巨噬细胞集落刺激因子抗体引起的。正在积极开发替代粒细胞-巨噬细胞集落刺激因子或清除这些抗体的治疗靶点。目前的标准治疗方法是对这些患者进行全肺灌洗,以清除肺泡空间,帮助改善呼吸生理。本文报告了 1 例 PAP 病例,并对该罕见疾病的诊断和治疗进行了文献复习,旨在提高医生在治疗出现肺泡浸润的患者时的认识。

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