A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features

Abstract

Pulmonary alveolar proteinosis (PAP) is a lung disease characterized by accumulation of lipid-rich proteinaceous materials that stain positive with periodic acid-Schiff (PAS) in the pulmonary alveolar space [1,2]. Although autoimmune PAP, pulmonary asbestosis, and idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) are independent pulmonary disorders, cases of secondary PAP caused by asbestosis or PAP-leading pulmonary fibrosis may occur occasionally. Differential diagnosis among these diseases is important but difficult. Here we report a case of autoimmune PAP with pulmonary asbestosis-like features including asbestos bodies in the lung tissue and pleural thickening on high-resolution computed tomography (HRCT), and a histologically possible UIP pattern. CASE REPORT A 78-year-old man visited a local hospital in May 2016 because of persistent productive cough and ground-glass opacities (GGOs) were observed on a chest radiograph and HRCT at a nearby hospital in May 2016. The productive cough eventually resolved, but the chest radiograph and HRCT showed expansion