Rapamycin Treatment Of Diffuse Cystic Lung Disease:One Case Report And Literature Review

Objective: Combined with clinical data of rapamycin treatment of diffuse cystic lung disease,to explore the efficacy,side effect/ adverse,dose and course of treatment in the diffuse cystic lung disease with rapamycin.Methods: We retrospectively analyzed the clinical manifestations,laboratory examinations,CT image and pathology data of 1 case of diffuse cystic lung disease in our hospital,and reviewed the relevant literature to discuss the characteristics of rapamycin in the treatment of diffuse cystic lung disease.Results :(1)A middle age women in our hospital presented with a complaint of progressive dyspnea,cough and hemoptysis in a chronic course.She was accepted intercostal catheters draining chylous effusion from the two sides pleura and the thoracic catheter ligation.No smoking,no special drug application history,no similar family history in the past.Chest HRCT showed diffuse cystic lesions with glass change and pneumothorax.Pulmonary function showed obstructive and restrictive lung function.Autoimmune antibody detection was negative.Without the multiple system damage such as joint pain,rash,kidney damage,anemia and so on.Combined with TBLB biopsy andimmunohistochemistry results,it most likely was pulmonary lymphangiomyomatosis,but can’t exclude pulmonary langerhans cell histiocytosis.Given oral prednisone therapy,the patient’s symptoms and the cystic lesions worsened,therefore rapamycin(2mg/day)as the new therapy.After 13 months therapy,the symptom,pulmonary function,cystic lesions of patient was better than before.Then she withdrawed all medicine 1 month ago,and the illness is stable.(2)Literature review results: A total of 39 cases of literature were collected,including 4 cases in China and 35 cases in foreign countries.All were female,and the mean age was 35±8.3 years old.The main manifestations were progressive dyspnea(64.1%),cough(23.1%),chylothorax(41.0%),pneumothorax(28.2%).8 cases’ blood gas analysis showed hypoxemia.The lung function mainly manifested as obstructive dysfunction and diffusion dysfunction.Chest HRCT showed diffuse cystic lesions in both lungs.All patients were given therapy for rapamycin.The symptoms of dyspnea,cough and so on were alleviated.Pneumothorax and chylous effusion were absorbed,and cystic lesions in the lungs partially decreased.But it can present side effects such as oral ulcers and hyperlipidemia and so on.Conclusions: Rapamycin can better symptoms,reduce cystic lesions in diffuse cystic lung disease,but there are treatment side effects.