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Thorax Online First, published on October 9, 2017 as 10.1136/thoraxjnl-2017-210872 Orphan lung disease
Original Article
Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study Janet Bee,1 Sharon Fuller,1 Suzanne Miller,2 Simon R Johnson1,2 1
Abstract 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 Correspondence to phenotyping, serial lung function, rapamycin and safety Professor Simon R Johnson, Division of Respiratory Medicine, monitoring were performed according to a clinical University of Nottingham, protocol. Lung function change, measured as FEV1 slope Respiratory Medicine, Queens ), was reported for those treated for 1 year or (ΔFEV 1 Medical Centre, Nottingham NG7 2UH, UK; simon.johnson@ longer. Results Rapamycin was associated with improved nottingham.ac.uk ΔFEV1 in 21 individuals where pretreatment data Received 7 August 2017 were available (p
Thorax Online First, published on October 9, 2017 as 10.1136/thoraxjnl-2017-210872 Orphan lung disease
Original Article
Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study Janet Bee,1 Sharon Fuller,1 Suzanne Miller,2 Simon R Johnson1,2 1
Abstract 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 Correspondence to phenotyping, serial lung function, rapamycin and safety Professor Simon R Johnson, Division of Respiratory Medicine, monitoring were performed according to a clinical University of Nottingham, protocol. Lung function change, measured as FEV1 slope Respiratory Medicine, Queens ), was reported for those treated for 1 year or (ΔFEV 1 Medical Centre, Nottingham NG7 2UH, UK; simon.johnson@ longer. Results Rapamycin was associated with improved nottingham.ac.uk ΔFEV1 in 21 individuals where pretreatment data Received 7 August 2017 were available (p
