Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study.
Yongzhong ZhanLisha ShenWenshuai XuXiuxiu WuWeihong ZhangJun WangXue LiYanli YangXinlun TianKai-Feng XuPublished in: Orphanet journal of rare diseases (2018)
Long-term use of sirolimus is safe in patients with LAM. LAM patients with FEV1 less than 70% predicted and symptomatic chylothorax are suitable for receiving sirolimus therapy. The maintaining serum trough levels of sirolimus are recommended between 5 to 9.99 ng/mL.