Abstract：Objective To summarize the document literature of pulmonary lymphangioleiomyomatosis (PLAM) in Chinese population in the last thirty years,and to analyze the clinical and pathological features,treatment progress and prognosis of PLAM.Methods Through Wanfang,Weipu Chinese database and China Biology Medicine database (CBM),the document literature of PLAM from January 1986 to April 2020 were retrieved and analyzed.Results A total of 150 articles were included in this study,among 289 patients,283 cases (97.9%) were females and 6 cases (2.1%) were males.Dyspnea was the most common clinical manifestation (90 cases,31.1%),followed by shortness of breath,cough,chest tightness and haemoptysis.The most common extrapulmonary manifestation or complication was pneumothorax (78 cases,66.1%).The most common chest imaging manifestation was multiple different sized thin-walled cystic shadows in whole lung (278 cases,96.2%),followed by pneumothorax (64 cases,23.0%) and pleural effusion (49 cases,17.6%).There were 243 cases mentioned the diagnostic method.The diagnosis was confirmed by pathology (241 cases,99.2%),followed by typical HRCT findings with increased vascular endothelial growth factor-D (VEGF-D) (2 cases,0.8%).Pathological features showed that the lung tissue was destroyed,the alveoli expand and fuse with each other to form bullae,and the abnormal smooth muscle cell proliferation in the lung interstitium,mainly fusiform and epithelioid,distributed around lymphatic vessels and blood vessels.There were 158 cases with immunohistochemical results mentioned in the literature.The most common positive result was human melanoma monoclonal antibody-45 (HMB-45) (123 cases,77.8%),followed by smooth muscle actin (SMA) (118 cases).There were 157 patients mentioned the initial diagnosis,the misdiagnosis rate was 63.1%,and the most misdiagnosed was pulmonary interstitial fibrosis (26 cases,16.6%).In terms of treatment,targeted therapies have gradually increased,and the effects have been remarkable.There were 18 cases of lung transplantation were treated with effective treatment.Conclusion PLAM is a multi-organ disease.PLAM occurs commonly in child-bearing age women,the most common manifestation is progressive dyspnea,the imaging and pathological manifestation is characteristic.Compared with invasive diagnosis methods such as bronchoscopy,thoracoscopy,and open-thoracic lung biopsy,VEGF-D will be more widely used for diagnosis and assessment of disease in future work.At present,targeted therapy and lung transplantation are effective treatment methods.
刘佳；李玉荣. 肺淋巴管平滑肌瘤病临床特点及诊疗现状[J]. 中国当代医药, 2020, 27(35): 64-68.
LIU Jia ；LI Yu-rong. Clinical features and current status of diagnosis and treatment of pulmonary lymphangioleiomyomatosis. 中国当代医药, 2020, 27(35): 64-68.
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