Iftikhar Hira, Nair Girish B, Kumar Anupam
Division of Pulmonary and Critical Care, Beaumont Health, OUWB School of Medicine, Royal Oak, MI, USA.
Division of Pulmonary and Critical Care, Baylor College of Medicine, Houston, TX, USA.
Ther Clin Risk Manag. 2021 Aug 10;17:701-710. doi: 10.2147/TCRM.S193884. eCollection 2021.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary surfactant homeostasis disorder resulting in buildup of lipo-proteinaceous material within the alveoli. PAP is classified as primary (autoimmune and hereditary), secondary, congenital and unclassifiable type based on the underlying pathogenesis. PAP has an insidious onset and can, in some cases, progress to severe respiratory failure. Diagnosis is often secured with bronchoalveolar lavage in the setting of classic imaging findings. Recent insights into genetic alterations and autoimmune mechanisms have provided newer diagnostics and treatment options. In this review, we discuss the etiopathogenesis, diagnosis and treatment options available and emerging for PAP.
肺泡蛋白沉积症（PAP）是一种罕见的肺表面活性物质稳态紊乱疾病，可导致肺泡内脂蛋白样物质积聚。根据潜在发病机制，PAP可分为原发性（自身免疫性和遗传性）、继发性、先天性和无法分类型。PAP起病隐匿，在某些情况下可进展为严重呼吸衰竭。在典型影像学表现的背景下，通常通过支气管肺泡灌洗来确诊。对基因改变和自身免疫机制的最新认识提供了新的诊断和治疗选择。在本综述中，我们讨论了PAP的病因发病机制、现有及新出现的诊断和治疗选择。
