Abstract: Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease characterized by diffuse cystic lesions that primarily affects women of reproductive ages and leads to respiratory failure at the end stage of the disease, and significantly impacts patients' quality of life. The clinical use of mammalian target of rapamycin (mTOR) inhibitors (e.g. sirolimus) has moderated the rate of disease progression and significantly improved the survival in LAM patients. During the clinical diagnosis, treatment, and follow-up of LAM disease, the clinical characteristics and the course of the disease of different LAM patients are heterogeneous which might suggest differences in disease progression and long-term prognosis. The present review summarizes the progress of research on risk factors for LAM progression and mortality in order to optimize individualized intervention protocols in clinical practice and to improve long-term prognosis of patients.

Key words: lymphangioleiomyomatosis, pulmonary function, vascular endothelial growth factor D (VEGF-D), sirolimus