TY - JOUR T1 - Pulmonary alveolar proteinosis JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 98 LP - 107 DO - 10.1183/09059180.00001311 VL - 20 IS - 120 AU - R. Borie AU - C. Danel AU - M-P. Debray AU - C. Taille AU - M-C. Dombret AU - M. Aubier AU - R. Epaud AU - B. Crestani Y1 - 2011/06/01 UR - http://err.ersjournals.com/content/20/120/98.abstract N2 - 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. ER -