Therapeutic Bronchoscopic Segmental/Lobar Lavage for Unilateral Pulmonary Alveolar Proteinosis-A Case Report

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by an accumulation of periodic acid-Schiff-positive materials in the alveolar space. The typical image findings are bilateral diffuse interstitial and parenchymal infiltrates shown on chest radiographs and/ or computed tomography (CT) of the chest. We report a case of unilateral PAP in a 51-year-old woman with unilateral emphysema. Chest radiographs and thoracic CT demonstrated findings that were highly suggestive of PAP in the right lung and emphysema in the left lung. Bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) were performed and a diagnosis of idiopathic PAP (iPAP) was made based on the gross appearance of retrieved BAL fluid (BALF), cytological examination of the BALF, and the pathologic findings of TBLB specimens. Because the patient had significant hypoxemia, therapeutic bronchoscopic segmental-lobar lavage was performed under sedation and endotracheal tube intubation. After 4 cycles of lobar lavage, the clinical symptoms improved remarkably and the patient was discharged and followed up at the outpatient department. Therapeutic bronchoscopic segmental/lobar lavage may be effective in patients with iPAP, especially in those who are at high risk for therapeutic whole lung lavage.