A Case of Pulmonary Alveolar Proteinosis Associated With Silicosis

진폐와 병발된 폐포단백증 1예

Pulmonary alveolar proteinosis is characterized by the accumulation of PAS positive lipoproteinaceous or amorphous proteinaceous material in the alveolar space with spared delicate septal architecture of the lung interstitium and impaired gas exchange of alveoli. We experienced a case of secondary pulmonary alveolar proteinosis in a 41 year old male patient who have occupational history of engagement as a mason over 4year. He compalined exertional dyspnea and chest discomfort, and presented fine inspiratory crackle at both lower lung field, numerous fine nodular denisties in both lung field with peripheral sparing. Light microscopic finding of lung tissue obtained by transbronchiallung biopsy revealed homogenous eosinophilic colloid-like luminal content in the alveolar space, and electron microscopy of bronchoalveolar lavage fluid concentrate showed electron-dense multilamellated structures. To treat the disease, we tried whole lung lavage of right lung with isotonic saline under general anesthesia. After whole lung lavage of right lung, he showed markid improvement of symptom and partial improvement of chest X-ray findings. The patient has been followed for 12 month until now, with no evidence of aggravation.

노작성 호흡곤란과 흉부 X-선 사진상의 이상소견으로 입원한 환자에서 직업력상의 규사폭로력과 조직검사에서의 폐포단백증의 소견으로 이차성 폐포단백증을 진단하고 전폐세척술의 시행으로 증상의 호전을 보인 1 예를 경험하였기에 문헌고찰과 함께 보고하였다.