PDF(Pubmed)
Abstract：
Pulmonary alveolar proteinosis (PAP) is an ultra-rare disease caused by impaired pulmonary surfactant clearance due to the dysfunction of alveolar macrophages or their signaling pathways. PAP is categorized into autoimmune, congenital, and secondary PAP, with autoimmune PAP being the most prevalent. This article aims to present a comprehensive review of PAP classification, pathogenesis, clinical presentation, diagnostics, and treatment. The literature search was conducted using the PubMed database and a total of 67 articles were selected. The PAP diagnosis is usually based on clinical symptoms, radiological imaging, and bronchoalveolar lavage, with additional GM-CSF antibody tests. The gold standard for PAP treatment is whole-lung lavage. This review presents a summary of the most recent findings concerning pulmonary alveolar proteinosis, pointing out specific features that require further investigation.
摘要：
肺泡蛋白沉积症（PAP）是由于肺泡巨噬细胞或其信号通路功能障碍而导致的肺表面活性物质清除受损而引起的一种罕见疾病。PAP分为自身免疫，先天性,和次要PAP，以自身免疫性PAP最为普遍。本文旨在对PAP分类进行全面综述，发病机制，临床表现,诊断,和治疗。使用PubMed数据库进行文献检索,共筛选67篇文献。PAP诊断通常基于临床症状，放射成像，支气管肺泡灌洗,额外的GM-CSF抗体测试。PAP治疗的金标准是全肺灌洗。这篇综述总结了有关肺泡蛋白沉积症的最新发现，指出需要进一步调查的具体特征。
