Summary of Recommendations

Document Overview

Design and created by Guideline Central in participation with the European Respiratory Society.

Diagnosis and Management of Pulmonary Alveolar Proteinosis

European Respiratory Society

Publication Date: Aug 15, 2024

Page Last Updated: May 5, 2026

Grading of Recommendations, Assessment, Development, and Evaluation (GRADE)-Based Recommendations

Implications for:	Strong Recommendation (S)	Conditional Recommendation (C)
Patients	Most individuals in this situation would want the recommended course of action, and only a small proportion would not.	The majority of individuals in this situation would want the suggested course of action, but many would not.
Clinicians	Most individuals should receive the intervention. Adherence to this recommendation according to the guideline could be used as a quality criterion or performance indicator. Formal decision aids are not likely to be needed to help individuals make decisions consistent with their values and preferences.	Recognize that different choices will be appropriate for individual patients and that you must help each patient arrive at a management decision consistent with his or her values and preferences. Decision aids may be useful in helping individuals to make decisions consistent with their values and preferences.
Policy	The recommendation can be adopted as policy in most situations.	Policymaking will require substantial debate and involvement of various stakeholders.

Certainty in the Evidence
High	H	Low	L
Moderate	M	Very Low	VL

Source: Grading of Recommendations Assessment, Development and Evaluation Working Group (Schunemann HJ et al. Am J Respir Crit Care Med. 2006;174:605-14. Guyatt GH et al. BMJ 2008;336:924-6).

Document Overview

Document Title: Diagnosis and Management of Pulmonary Alveolar Proteinosis

Authoring Society: European Respiratory Society

Document Publication Date: Aug 15, 2024

Page Last Reviewed/Updated: May 5, 2026

Document Type: Guideline

Country of Publication: European

Full Text Freely Available: Yes

Full Text Guideline: erj.ersjournals.com/content/early/2024/08/08/13993003.00725-2024

Source Citation: McCarthy C, Bonella F, O'Callaghan M, et al. European Respiratory Society guidelines for the Diagnosis and Management of Pulmonary Alveolar Proteinosis. Eur Respir J 2024; in press (https://doi.org/10.1183/13993003.00725-2024).

Supplemental Implementation Resources

Video

Document Scope, Criteria, and Use Cases

Document Objectives: Pulmonary alveolar proteinosis (PAP) is a rare syndrome caused by several distinct diseases leading to progressive dyspnoea, hypoxemia, 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, evaluation of antibodies against granulocyte macrophage colony stimulating factor (GM-CSF), genetic testing, and, eventually, lung biopsy. The management options are focused at removing the proteinaceous material by whole lung lavage (WLL), augmentation therapy with GM-CSF, rituximab, plasmapheresis, and lung transplantation. The presented diagnostic and management guideline aim to provide guidance to physicians managing patients with PAP.

Scope: Assessment and Screening, Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D011649 - Pulmonary Alveolar Proteinosis

Keywords: Pulmonary alveolar proteinosis

Inclusion Criteria: Male, Female, Adult, Older Adult

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

PICO Questions

When should patients with clinical and radiological features consistent with a diagnosis of PAP undergo bronchoalveolar lavage (BAL)?
When should patients with clinical and radiological features consistent with a diagnosis of PAP undergo lung biopsy for histologic analysis?
When should patients with clinical and radiological features consistent with PAP undergo GM-CSF antibody testing for diagnosing autoimmune PAP?
In patients with clinical symptoms and/or functional impairment due to PAP should whole lung lavage be used versus to no whole lung lavage?
In patients with confirmed autoimmune PAP should exogenous GM-CSF be used versus no exogenous GM-CSF?
In patients with confirmed autoimmune PAP should rituximab be used versus no immunosuppressive treatment?
In patients with confirmed autoimmune PAP should plasmapheresis be used versus no plasmapheresis?

Number of Source Documents: 118

Literature Search Start Date: Thursday, May 19, 2022

Literature Search End Date: Tuesday, August 9, 2022

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: Yes

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses funding source?: Yes

Discloses conflicts of interest?: Yes

Includes benefits/harms analysis with recommendations?: Yes