Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis.

ABSTRACT:

Introduction

The Multicenter International Lymphangioleiomyomatosis (LAM) Efficacy of Sirolimus (MILES) trial revealed that sirolimus stabilised lung function in patients with moderately severe LAM. The purpose of this study was to further examine the MILES cohort for the effects of racial, demographic, clinical and physiological patient characteristics on disease progression and treatment response in LAM.

Methods

MILES subjects were stratified on the basis of menopausal status (pre-menopausal/post-menopausal), race (Asian/Caucasian), bronchodilator responsiveness (present/absent), initial forced expiratory volume in 1 s (FEV₁; 51-70% versus ≤50% predicted) and tuberous sclerosis complex (TSC) association (yes/no). A linear mixed effects model was used to c

SUBMITTER: Gupta N 

PROVIDER: S-EPMC7394189 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature