Abstract: Lymphangioleiomyomatosis (LAM) is a rare systemic disease mainly affecting reproductive women. LAM is generally aggressive in nature and ultimately results in respiratory failure. The disease spreads through lymphatic vessels, and the lung is the main target organ, leading to cystic remodeling of the lung. In the last few years, studies have enhanced the understanding of LAM pathogenesis. In addition to the over activation of mTOR pathway, NR2F2 mutation is also a possible pathogenesis. The research and development of some new imaging technologies may provide more safe means for evaluation of efficacy and follow-up. Although rapamycin, the mTOR inhibitor, has shown some benefit in LAM patients, which can stabilize lung function and improve quality of life, the disease continues to progress after the cessation of treatment. Immunotherapy may provide a new treatment direction for LAM. This review will focus on the new progress in pathogenesis and treatment of LAM. 

Key words: lymphangiomyomatosis, pathogenesis, pathology, tomography, X-ray computer