Abstract: Objective Pulmonary alveolar proteinosis (PAP) is a rare chronic interstitial lung disease characterized by accumulation of phospholipid protein-like substances in alveoli and bronchioles. At present, the clinical treatment of PAP in China is still whole lung lavage. Basic and clinical trials at home and abroad have found that PAP is not only related to GM-CSF autoantibodies, but also related to CSF2ra, CSF2rb, SFTPB gene mutations and targeted macrophage cholesterol homeostasis (peroxisome proliferator-activated receptor, PPARγ).With the progress of the research on the genetic level of the disease, the corresponding progress in diagnosis and treatment of the disease have also be made. In addition to pathological examination of lung biopsy, serum GM-CSF autoantibody detection, serum GM-CSF level detection and genetic testing have also got progress and are widely used in clinical practice. Recently, inhalation or subcutaneous injection of GM-CSF, rituximab, pioglitazone, statins, gene therapy, and plasma exchange have become research hots. This paper reviewed these new diagnosis and treatment methods of PAP.