Diagnosis and Management of Pulmonary Alveolar Proteinosis

Publication Date: August 15, 2024
Last Updated: August 16, 2024

Summary of Recommendations

We recommend that BAL be performed as part of the diagnostic work up of patients with suspected PAP. BAL should include differential cell count, periodic-acid-Schiff (PAS)-staining, and microbiology. (S, VL )
620
We suggest to not routinely perform lung biopsy as part of the diagnostic work up of patients with suspected PAP. (C, M )
620
We recommend GM-CSF antibody testing for diagnosing autoimmune PAP for all patients with suspected or confirmed PAP syndrome. (S, M )
620
We recommend performing bilateral whole lung lavage in patients with autoimmune PAP with evidence of gas exchange impairment and either symptoms, or functional impairment. (S, VL )
620
No recommendation for or against whole lung lavage in other PAP types can be made due to lack of evidence. We suggest seeking advice from an expert centre on an individual case basis. (, )
620
We recommend inhaled GM-CSF for symptomatic patients with confirmed autoimmune PAP. (S, VL )
620
We suggest the use of rituximab for patients with confirmed autoimmune PAP who remain symptomatic, requiring supplemental oxygen, despite whole lung lavage therapy or exogenous GMCSF treatment. (C, VL )
620
We suggest the use of plasmapheresis for patients with confirmed autoimmune PAP who remain symptomatic, requiring high flow of supplemental oxygen (≥4L /min) or two or more WLL over a period of a year, despite receiving exogenous GM-CSF and rituximab, or having previously failed these treatments. (C, VL )
620
We suggest lung transplantation for patients with PAP progressing despite whole lung lavage and/or pharmacological treatment, who fulfil the International Society for Heart and Lung Transplantation (ISHLT) criteria for patients with interstitial lung disease. (C, VL )
620

Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Diagnosis and Management of Pulmonary Alveolar Proteinosis

Authoring Organization

European Respiratory Society

Publication Month/Year

August 15, 2024

Last Updated Month/Year

August 27, 2024

Supplemental Implementation Tools

Document Type

Guideline

Country of Publication

European

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.

PICO Questions

  1. When should patients with clinical and radiological features consistent with a diagnosis of PAP undergo bronchoalveolar lavage (BAL)?

  2. When should patients with clinical and radiological features consistent with a diagnosis of PAP undergo lung biopsy for histologic analysis?

  3. When should patients with clinical and radiological features consistent with PAP undergo GM-CSF antibody testing for diagnosing autoimmune PAP?

  4. In patients with clinical symptoms and/or functional impairment due to PAP should whole lung lavage be used versus to no whole lung lavage?

  5. In patients with confirmed autoimmune PAP should exogenous GM-CSF be used versus no exogenous GM-CSF?

  6. In patients with confirmed autoimmune PAP should rituximab be used versus no immunosuppressive treatment?

  7. In patients with confirmed autoimmune PAP should plasmapheresis be used versus no plasmapheresis?

Inclusion Criteria

Male, Female, Adult, Older adult

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D011649 - Pulmonary Alveolar Proteinosis

Keywords

Pulmonary alveolar proteinosis

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).

Methodology

Number of Source Documents
118
Literature Search Start Date
May 19, 2022
Literature Search End Date
August 9, 2022