We followed a patient with pulmonary alveolar proteinosis who received bronchopulmonary lavages for eleven years (seven times on the left, six times on the right). Massive bronchopulmonary lavage was performed under general anesthesia with ventilation of one lung. A solution of 0.9% NaCl equal in volume to the capacity of the lung was administered by volume limited instillation for each lavage. Symptoms improved after lavage, but the effects attenuated with each lavage. Interstitial shadowing and bullous change, the so-called honey-comb lung, gradually became worse in the bilateral lower lung fields. The patient died of respiratory failure due to pneumonia and bilateral pneumothorax eleven years after the first admission.
Microscopic examination revealed that the alveolar septum was almost normal at the first admission but had become infiltrated with inflammatory cells and had become markedly fibrous by the terminal stage. Arterial oxygen tension remained within the normal range, but carbon monoxide diffusing capacity (DL_CO) decreased as the patient's condition worsened. We believe DL_CO is important for following the disease. Improvement in DL_CO was observed for several months after lavage, but subsequently became worse. Changes in DL_CO may be useful to determine the timing of the next bronchopulmonary lavage in pulmonary alveolar proteinosis.