Open AccessReduced Intestinal Inflammation With Lumacaftor/Ivacaftor in Adolescents With Cystic Fibrosis
Author(s) -
Tétard Candice,
Mittaine Marie,
Bui Stéphanie,
Beaufils Fabien,
Maumus Pascale,
Fayon Michael,
Burgel PierreRegis,
Lamireau Thierry,
Delhaes Laurence,
Mas Emmanuel,
Enaud Raphaël
Publication year - 2020
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000002864
Subject(s) - ivacaftor , cystic fibrosis , medicine , cystic fibrosis transmembrane conductance regulator , inflammation , calprotectin , gastroenterology , inflammatory bowel disease , disease
A chronic intestinal inflammation may occur in patients with cystic fibrosis (CF), while no therapeutic management is proposed. Although Lumacaftor/Ivacaftor is well‐known to modulate the defective cystic fibrosis transmembrane conductance regulator (CFTR) protein in lungs, no data are available on the impact of this treatment on CF intestinal disorders. We, therefore, investigated the evolution of intestinal inflammation after initiation of Lumacaftor/Ivacaftor in CF adolescents (median of follow‐up: 336 days [IQR: 278;435]). Median fecal calprotectin concentrations decreased significantly after Lumacaftor/Ivacaftor initiation (102 μg/g [IQR: 69–210]) compared with the baseline (713 μg/g (IQR:148–852), P = 0.001). To our knowledge, this study showed for the first time that CF‐related intestinal inflammation is improved by Lumacaftor/Ivacaftor treatment.