European Respiratory Society guidelines for the diagnosis and management of pulmonary alveolar proteinosis.

IF 16.6 1区 医学 Q1 RESPIRATORY SYSTEM
European Respiratory Journal Pub Date : 2024-11-14 Print Date: 2024-11-01 DOI:10.1183/13993003.00725-2024
Cormac McCarthy, Francesco Bonella, Marissa O'Callaghan, Clairelyne Dupin, Tiago Alfaro, Markus Fally, Raphael Borie, Ilaria Campo, Vincent Cottin, Aurelie Fabre, Matthias Griese, Alice Hadchouel, Stephane Jouneau, Maria Kokosi, Effrosyni Manali, Helmut Prosch, Bruce C Trapnell, Marcel Veltkamp, Tisha Wang, Ingrid Toews, Alexander G Mathioudakis, Elisabeth Bendstrup
{"title":"European Respiratory Society guidelines for the diagnosis and management of pulmonary alveolar proteinosis.","authors":"Cormac McCarthy, Francesco Bonella, Marissa O'Callaghan, Clairelyne Dupin, Tiago Alfaro, Markus Fally, Raphael Borie, Ilaria Campo, Vincent Cottin, Aurelie Fabre, Matthias Griese, Alice Hadchouel, Stephane Jouneau, Maria Kokosi, Effrosyni Manali, Helmut Prosch, Bruce C Trapnell, Marcel Veltkamp, Tisha Wang, Ingrid Toews, Alexander G Mathioudakis, Elisabeth Bendstrup","doi":"10.1183/13993003.00725-2024","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6000,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Respiratory Journal","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1183/13993003.00725-2024","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/11/1 0:00:00","PubModel":"Print","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
引用次数: 0

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

欧洲呼吸学会肺泡蛋白病诊断和管理指南。
背景: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,会导致进行性呼吸困难、低氧血症、呼吸衰竭风险以及因蛋白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、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部活检。治疗方案主要是通过全肺灌洗(WLL)、GM-CSF 增强疗法、利妥昔单抗、浆细胞吸出术和肺移植清除蛋白物质。本诊断和管理指南旨在为管理 PAP 患者的医生提供指导:欧洲呼吸学会特别工作组委员会由临床医师、方法学专家和具有 PAP 经验的患者组成,该委员会根据《欧洲呼吸学会临床实践指南手册》和 GRADE(建议、评估、发展和评价分级)方法制定了相关建议。这包括对文献进行系统性回顾,并应用 GRADE 方法评估证据的确定性和建议的力度。委员会提出了五个 PICO(患者、干预、比较、结果)问题和两个叙述性问题,以制定具体的循证建议:特别小组委员会针对五个 PICO 问题提出了建议。结果:专责小组委员会针对五个 PICO 提出了建议,包括 PAP 与 WLL 的管理、GM-CSF 增强疗法、利妥昔单抗、血浆置换术和肺移植。此外,委员会还根据叙述性问题就 GM-CSF 抗体检测、诊断性支气管肺泡灌洗(BAL)和活组织检查的使用提出了建议:结论:PAP 的诊断基于 CT 和 BAL 细胞学或肺组织学,而 PAP 特定致病疾病的诊断则需要 GM-CSF 抗体检测或基因分析。目前有多种疗法可用于治疗 PAP,包括 WLL 和 GM-CSF 增强疗法,但支持性证据仍然有限。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
European Respiratory Journal
European Respiratory Journal 医学-呼吸系统
CiteScore
27.50
自引率
3.30%
发文量
345
审稿时长
2-4 weeks
期刊介绍: The European Respiratory Journal (ERJ) is the flagship journal of the European Respiratory Society. It has a current impact factor of 24.9. The journal covers various aspects of adult and paediatric respiratory medicine, including cell biology, epidemiology, immunology, oncology, pathophysiology, imaging, occupational medicine, intensive care, sleep medicine, and thoracic surgery. In addition to original research material, the ERJ publishes editorial commentaries, reviews, short research letters, and correspondence to the editor. The articles are published continuously and collected into 12 monthly issues in two volumes per year.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信