BỆNH TÍCH PROTEIN PHẾ NANG PHỔI - TỔNG QUAN TÀI LIỆU VÀ BÁO CÁO CHÙM CA BỆNH

Abstract

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by the accumulation of surfactant in the alveoli and in airway macrophages, eventually leading to failure respiratory. PAP is part of a wide range of disturbances in surfactant balance (production and clearance). The classification of PAP includes 3 basic groups: (1) Primary/Autoimmune alveolar proteinosis; (2) Secondary alveolar proteinosis; (3) Congenital alveolar proteinosis. In which group (1) accounts for over 90% of cases. PAP syndrome can be definitively diagnosed based on history, clinical symptoms, computed tomography images, lung lavage testing; sometimes open lung biopsies. Whole lung lavage is the current standard therapy, and adjuvant pharmacological therapies are being investigated. We report 10 cases of PAP diagnosed and treated at the National Lung Hospital, from July 2020 to May 2022, especially emphasizing the analysis of chest computed tomography images, thereby helping to colleagues have a better approach to diagnostic imaging when encountering this disease.