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Research progress of diagnosis and treatment of pulmonary alveolar proteinosis
Received:March 29, 2022  Revised:April 26, 2022  Click here to download the full text
Citation of this paper:WANG Jia-qi,HAI Bing,YANG Yuan.Research progress of diagnosis and treatment of pulmonary alveolar proteinosis[J].Chinese Journal of Clinical Medicine,2022,29(4):696-700
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Author NameAffiliationE-mail
WANG Jia-qi Department of Respiratory and Critical Care Medicine, the Second Affiliated Hospital of Kunming Medical University, Kunming 650000, Yunnan, China  
HAI Bing Department of Respiratory and Critical Care Medicine, the Second Affiliated Hospital of Kunming Medical University, Kunming 650000, Yunnan, China 1910645481@qq.com 
YANG Yuan Department of Respiratory and Critical Care Medicine, the Second Affiliated Hospital of Kunming Medical University, Kunming 650000, Yunnan, China  
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.
keywords:pulmonary alveolar proteinosis  granulocyte-macrophage colony stimulating factor  diagnosis  treatment
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