A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis

Annals of the American Thoracic Society
Rahul G ArgulaMILES Study Investigators

Abstract

The Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus (MILES) trial demonstrated that sirolimus stabilized lung function and improved measures of functional performance and quality of life in patients with lymphangioleiomyomatosis. The physiologic mechanisms of these beneficial actions of sirolimus are incompletely understood. To prospectively determine the longitudinal computed tomographic lung imaging correlates of lung function change in MILES patients treated with placebo or sirolimus. We determined the baseline to 12-month change in computed tomographic image-derived lung volumes and the volume of the lung occupied by cysts in the 31 MILES participants (17 in sirolimus group, 14 in placebo group) with baseline and 12-month scans. There was a trend toward an increase in median expiratory cyst volume percentage in the placebo group and a reduction in the sirolimus group (+2.68% vs. +0.97%, respectively; P = 0.10). The computed tomographic image-derived residual volume and the ratio of residual volume to total lung capacity increased more in the placebo group than in the sirolimus group (+214.4 ml vs. +2.9 ml [P = 0.054] and +0.05 ml vs. -0.01 ml [P = 0.0498], respectively). A Markov transiti...Continue Reading

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Citations

May 1, 2019·Annals of the American Thoracic Society·Laura L WalkupJason C Woods
Mar 11, 2016·Annals of the American Thoracic Society·George R Washko, Grace Parraga
Nov 30, 2019·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Alicia ChenChi Wan Koo
Nov 25, 2017·Expert Review of Respiratory Medicine·Julie NijmehElizabeth P Henske
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Oct 9, 2018·Journal of the American Academy of Dermatology·Pablo García-MonteroÁngel Vera-Casaño
Jul 3, 2019·Respiratory Medicine and Research·D LecuelleV Jounieaux

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