Autoimmune pulmonary alveolar proteinosis and anti-GM-CSF antibody
10.3760/cma.j.cn114452-20240315-00141
- VernacularTitle:自身免疫性肺泡蛋白沉积症与抗GM-CSF抗体
- Author:
Xinhe ZHANG
1
;
Kaifeng XU
Author Information
1. 中国医学科学院北京协和医院呼吸与危重症医学科 疑难重症及罕见病全国重点实验室，北京 100730
- Keywords:
Pulmonary alveolar proteinosis;
Autoimmune pulmonary alveolar proteinosis;
Macrophages, alveolar;
Granulocyte macrophage colony-stimulating factor
- From:
Chinese Journal of Laboratory Medicine
2024;47(9):987-992
- CountryChina
- Language:Chinese
-
Abstract:
Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by dyspnea due to abnormal deposition of pulmonary surfactant in alveolar macrophages and alveoli, among which autoimmune PAP (aPAP) is the most common clinical type, accounting for about 90%. The abnormal production of anti-granulocyte-macrophage colony stimulating factor antibody (anti-GM-CSF antibody) leads to the dysfunction of alveolar macrophages. We summarize the current state of research on the pathogenesis of aPAP and describe the progress in the diagnosis and treatment of aPAP by discussing the abnormalities of signal pathway, dysfunction of alveolar macrophages and the production of anti-GM-CSF antibody.
