A Case of Autoimmune Pulmonary Alveolar Proteinosis That Improved After Segmental Bronchoalveolar Lavage Therapy, Including the Region Which Had Not Been Treated

Abstract

Background. Pulmonary alveolar proteinosis (PAP) is caused by abnormal intra-alveolar accumulation of surfactant protein, and the treatment of PAP is whole-lung lavage. Case. A 47-year-old Japanese man started to have progressive exertional dyspnea and nonproductive cough, and the diagnosis of PAP was made according to radiological, laboratory and pathological findings of bronchoalveolar lavage (BAL) fluid and transbronchial lung biopsy. Then he was admitted to our hospital for PAP treatment. Lingular segmental and subsequent right middle lobe BAL therapy were performed. Chest roentgenogram and computed tomography (CT) showed an improvement of bilateral lower lobes that had not been treated in addition to the treated areas. Conclusion. Segmental BAL therapy was effective not only in lavaged areas but also non-lavaged regions, therefore, segmental BAL therapy can be an alternative treatment modality in addition to total lung lavage therapy for treating patients with PAP.