Pulmonary alveolar proteinosis (PAP) is a rare syndrome caused by several distinct diseases leading to progressive dyspnoea, hypoxaemia, risk of respiratory failure and early death due to accumulation of proteinaceous material in the lungs. Diagnostic strategies may include computed tomography (CT) of the lungs, bronchoalveolar lavage (BAL), evaluation of antibodies against granulocyte–macrophage colony-stimulating factor (GM-CSF), genetic testing and, eventually, lung biopsy. The management options are focused on removing the proteinaceous material by whole lung lavage (WLL), augmentation therapy with GM-CSF, rituximab, plasmapheresis and lung transplantation. The presented diagnostic and management guidelines aim to provide guidance to physicians managing patients with PAP.

A European Respiratory Society Task Force composed of clinicians, methodologists and patients with experience in PAP developed recommendations in accordance with the ERS Handbook for Clinical Practice Guidelines and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. This included a systematic review of the literature and application of the GRADE approach to assess the certainty of evidence and strength of recommendations. The Task Force formulated five PICO (Patients, Intervention, Comparison, Outcomes) questions and two narrative questions to develop specific evidence-based recommendations.

The Task Force developed recommendations for the five PICO questions. These included management of PAP with WLL, GM-CSF augmentation therapy, rituximab, plasmapheresis and lung transplantation. Also, the Task Force made recommendations regarding the use of GM-CSF antibody testing, diagnostic BAL and biopsy based on the narrative questions. In addition to the recommendations, the Task Force provided information on the hierarchy of diagnostic interventions and therapy.

The diagnosis of PAP is based on CT and BAL cytology or lung histology, whereas the diagnosis of specific PAP-causing diseases requires GM-CSF antibody testing or genetic analysis. There are several therapies including WLL and augmentation therapy with GM-CSF available to treat PAP, but supporting evidence is still limited.

肺泡蛋白沉积症 （PAP） 是一种罕见的综合征，由几种不同的疾病引起，导致进行性呼吸困难、低氧血症、呼吸衰竭风险和由于蛋白质物质在肺部积聚而过早死亡。诊断策略可能包括肺部计算机断层扫描 （CT）、支气管肺泡灌洗 （BAL）、粒细胞-巨噬细胞集落刺激因子 （GM-CSF） 抗体评估、基因检测以及最终的肺活检。管理方案的重点是通过全肺灌洗 （WLL）、GM-CSF 增强疗法、利妥昔单抗、血浆置换和肺移植去除蛋白质物质。本报告和管理指南旨在为管理 PAP 患者的医生提供指导。

由临床医生、方法学家和具有 PAP 经验的患者组成的欧洲呼吸学会工作组根据 ERS 临床实践指南手册和 GRADE（建议分级、评估、开发和评估）方法制定了建议。这包括对文献的系统评价和GRADE方法的应用，以评估证据的质量和建议的强度。工作组制定了 5 个 PICO（患者、干预、比较、结果）问题和 2 个叙述性问题，以制定具体的循证建议。

工作组为 PICO 的 5 个问题制定了建议。这些治疗包括 WLL 的 PAP 管理、GM-CSF 增强治疗、利妥昔单抗、血浆置换和肺移植。此外，工作组还根据叙述性问题提出了关于使用 GM-CSF 抗体检测、诊断性 BAL 和活检的建议。除了建议外，工作组还提供了有关诊断干预和治疗层次结构的信息。

PAP 的诊断基于 CT 和 BAL 细胞学或肺组织学，而特定 PAP 引起疾病的诊断需要 GM-CSF 抗体检测或基因分析。有几种疗法可用于治疗 PAP，包括 WLL 和 GM-CSF 增强疗法，但支持证据仍然有限。