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Pulmonary Alveolar Proteinosis
Francesco Bonella1, Raphael Borie2
1Pneumology Department, Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; Ruhrlandklinik, Tüschener Weg 40, Essen 45239, Germany.
Clinics in Chest Medicine
|October 19, 2025
Summary
Pulmonary alveolar proteinosis (PAP) is a rare lung disease. Diagnosis involves identifying the cause, with autoantibodies confirming autoimmune PAP, guiding targeted treatments for better outcomes.
Area of Science:
- Pulmonology
- Rare diseases
- Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by surfactant accumulation in lung alveoli.
- This condition leads to significant respiratory insufficiency.
- PAP can stem from various underlying causes, necessitating a thorough diagnostic approach.
Purpose of the Study:
- To outline the diagnostic workup for Pulmonary alveolar proteinosis (PAP).
- To highlight methods for differentiating autoimmune PAP from other forms.
- To discuss current and future therapeutic strategies for PAP.
Main Methods:
- Diagnostic evaluation focused on identifying the specific cause of PAP.
- Serum analysis for the detection of granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies.
- Review of treatment outcomes including whole lung lavage, inhalational GM-CSF, and management of secondary causes.
Main Results:
- Detection of GM-CSF neutralizing autoantibodies in serum is key to confirming autoimmune PAP.
- Whole lung lavage can improve alveolar function but does not offer a cure.
- Inhalational GM-CSF shows promise for autoimmune PAP, while treating underlying conditions is vital for secondary PAP.
Conclusions:
- Accurate diagnosis is crucial for effective PAP management.
- Targeted therapies, including inhalational GM-CSF and addressing underlying causes, improve patient outcomes.
- Genetic mutations causing PAP may benefit from future targeted treatments.
Keywords:
FibrosisGenetic mutationsGranulocyte-macrophage colony stimulating factor (GM-CSF)Pulmonary alveolar proteinosisSurfactant proteinsWhole lung lavageMore Related Videos
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