Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: A prospective national cohort study

Bee, Janet; Fuller, Sharon; Miller, Suzanne; Johnson, Simon R.

Abstract

© Article author(s) 2018. Rationale Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals. We examined lung function response and side effects to rapamycin in a national cohort. Methods Subjects were receiving rapamycin for progressive lung disease. Clinical evaluation, detailed phenotyping, serial lung function, rapamycin and safety monitoring were performed according to a clinical protocol. Lung function change, measured as FEV 1 slope (ΔFEV 1), was reported for those treated for 1 year or longer. Results Rapamycin was associated with improved ΔFEV 1 in 21 individuals where pretreatment data were available (p

Citation

Bee, J., Fuller, S., Miller, S., & Johnson, S. R. (2018). Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: A prospective national cohort study. Thorax, 73(4), 369-375. https://doi.org/10.1136/thoraxjnl-2017-210872

• Journal Article Type: Article
• Acceptance Date: Oct 2, 2017
• Online Publication Date: Oct 9, 2017
• Publication Date: Apr 1, 2018
• Deposit Date: Nov 8, 2017
• Publicly Available Date: Nov 8, 2017
• Journal: Thorax
• Print ISSN: 0040-6376
• Electronic ISSN: 1468-3296
• Publisher: BMJ Publishing Group
• Peer Reviewed: Peer Reviewed
• Volume: 73
• Issue: 4
• Pages: 369-375
• DOI: https://doi.org/10.1136/thoraxjnl-2017-210872
• Public URL: https://nottingham-repository.worktribe.com/output/886756
• Publisher URL: https://doi.org/10.1136/thoraxjnl-2017-210872
• Additional Information: Copyright ©2017 BMJ Publishing Group Ltd & British Thoracic Society