Abstract
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterized by the accumulation of surfactant-like phospholipoproteins in alveoli and interstitium that may result in respiratory failure. It affects mainly smokers.
Congenital form of the disease occurs in childhood, acquired forms occur in adulthood; the most common form is the autoimmune form, the secondary form accompanying other specified conditions is diagnosed less often. The diagnosis is based on a combination of high resolution computer tomography (HRCT), bronchoalveolar lavage (BAL) and histology obtained from trans-bronchial biopsy (TBLB) or open lung biopsy (OLB).
The main therapeutical method is a whole-lung lavage and the treatment of primary disease in the secondary forms. Lung transplantation is often the final solution predominantly in the congenital form.
The substitution with recombinant granulocytes stimulating factor and macrophages stimulating factor (GM-CSF) seems to be a promised therapeutical method in the autoimmune form. Smoking cessation is also important.