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[Pulmonary alveolar proteinosis].
S Jouneau1, M Kerjouan2, E Briens3
1Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université Rennes 1, 35043 Rennes, France.
Revue Des Maladies Respiratoires
|December 16, 2014
Summary
Alveolar proteinosis (AP) is a rare lung disease where surfactant builds up in the alveoli. Diagnosis involves CT scans and lavage, with treatments ranging from whole lung lavage to GM-CSF therapy for refractory cases.
Area of Science:
- Pulmonology
- Rare Diseases
- Immunology
Context:
- Alveolar proteinosis (AP) is a rare lung condition characterized by surfactant accumulation in alveoli, impairing gas exchange.
- AP presents in three forms: autoimmune (anti-GM-CSF antibodies), secondary (hematologic diseases, toxins, infections), and genetic (primarily in children).
- Diagnosis is suggested by CT scans showing interstitial lung disease with a 'crazy paving' pattern and confirmed by bronchoalveolar lavage.
Purpose:
- To outline the classification, diagnosis, and management of alveolar proteinosis.
- To highlight diagnostic criteria including imaging and lavage findings.
- To discuss current and emerging therapeutic strategies for AP.
Summary:
- AP involves alveolar surfactant buildup, classified into autoimmune, secondary, and genetic types.
- Diagnosis relies on CT scans and bronchoalveolar lavage; positive anti-GM-CSF antibodies suggest autoimmune etiology.
- Management includes whole lung lavage, with options like GM-CSF supplementation or rituximab for refractory cases.
Impact:
- Provides a comprehensive overview of alveolar proteinosis for clinicians and researchers.
- Emphasizes the importance of identifying the specific AP subtype for targeted treatment.
- Highlights the unpredictable clinical course and potential for spontaneous improvement, with a generally favorable 5-year survival rate of 95%.
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