Pulmonary Alveolar Proteinosis with a Rare Presentation: A Case Report

Chang-Wei Wu ； Yen-Lin, Huang ； Ching-Yao Yang

《胸腔醫學》 37卷2期 (2022/06) Pp. 96-102

摘要

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by a diffuse accumulation of lipoproteinaceous material in the distal airway and alveolar space that causes shortness of breath, hypoxia, respiratory failure and even death in severe cases. PAP could be a primary or secondary disease, and most PAP patients experienced progressive disease requiring treatment of the underlying disease with whole lung lavage or lung transplant. However, self-resolving PAP has also been described in a few case reports. Here, we reported the case of a 46-year-old man without known systemic disease or symptoms. Abnormal computed tomography imaging studies revealed a bilateral subpleural peri-bronchial crazy-paving pattern. Organized pneumonia was first suspected, but later, surgical resection confirmed a histological diagnosis of PAP. Partial regression of PAP was noted during the 7-month follow-up.

關鍵字

pulmonary alveolar proteinosis ； crazy-paving pattern ； ground glass opacity