Pulmonary alveolar proteinosis

• Main Authors: B. Crestani, R. Epaud, M. Aubier, M-C. Dombret, C. Taille, M-P. Debray, C. Danel, R. Borie
• Format: Article
• Language: English
• Published: European Respiratory Society 2011-06-01
• Series: European Respiratory Review
• Subjects: Granulocyte macrophage-colony stimulating factor; macrophage; pulmonary lavage; rituximab; surfactant
• Online Access: http://err.ersjournals.com/content/20/120/98.full.pdf+html

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic “crazy paving” pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.