Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis

Pulmonary alveolar proteinosis represents a syndrome with a number of possible aetiologies. The appearances on the HRCT scan of the thorax will often suggest the diagnosis and should be confirmed by examination of bronchoalveolar lavage fluid. Whole lung lavage appears to be the most effective form of treatment and is a safe technique in experienced hands. The overall prognosis is excellent with treatment. A few patients are resistant to whole lung lavage. It is important to investigate these non-responders further as they may respond to some of the more targeted treatments.