【摘要】 肺淋巴管肌瘤病(PLAM)是一种罕见的肺部疾病,好发于育龄期女性,主要临床表现为反复气胸、乳糜胸、呼吸困难等,典型影像学表现为两肺弥漫性囊性病变,治疗较困难,患者预后差。PLAM的治疗包括药物治疗、气胸及乳糜胸的处理、肺移植等,其中西罗莫司是主要的治疗药物。本文就PLAM的治疗研究进展进行综述,以期为临床治疗及后续研究提供参考。更多还原

【Abstract】 Pulmonary lymphangiomyomatosis is a rare lung disease that occurs in women of reproductive age. Main clinical manifestations are repeated pneumothorax, chylothorax, dyspnea and so on.Typical imaging manifestation is diffuse cystic lesions of both lungs. Clinical treatment is difficult, and prognosis is poor. Treatments for PLAM includes drugs treatment, managment of pneumothorax and chylothorax, and lung transplantation. Among them, Sirolimus is the main therapeutic drug. This article reviews the progress in treatment of PLAM to provide references for clinical treatment and future research of this disease.更多还原