Pulmonary alveolar proteinosis
Congenital pulmonary alveolar proteinosis — caused by mutations in the genes encoding surfactant protein B or C or the C chain of the receptor for granulocyte-macrophage colony-stimulating factor (GM-CSF). Treatment is supportive. Lung transplantation has been reported to be successful.
Secondary pulmonary alveolar proteinosis — due to impairment of macrophages in the lungs, secondary to some cancers, immunosuppression, inhalation of dust such as silica or toxic fumes, and certain infections. Therapy is directed at the underlying condition.
Acquired pulmonary alveolar proteinosis — an autoimmune disease in which the immune system attacks GM-CSF. (GM-CSF plays a critical role in the regulation of mature alveolar macrophages in the lung.) Acquired pulmonary alveolar proteinosis can be treated successfully by whole-lung lavage.
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