Assessment of efficacy and tolerability of elexacaftor-tezacaftor-ivacaftor in an observational cohort study of "aged" people with cystic fibrosis

Pulmonary Fibrosis

LONGITUDE: An observational study of the long-term effectiveness of elexacaftor/tezacaftor/ivacaftor in people aged ≥12 years with cystic fibrosis using data from the United Kingdom Cystic Fibrosis Registry - 2-year analysis

J Cyst Fibros

Vega-Hernandez G, MacGregor G, Wilfin A, Adams F, Haugh C, Baxter CA, Wöhling H, Clarke SL, Charman SC, Carr SB

doi: 10.1016/j.jcf.2025.04.012

Further Publications

Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies

Lancet Respir Med

Burgel PR, Da Silva J, Girodon E, Durieu I, Reynaud-Gaubert M, Murris-Espin M, Chiron R, Grenet D, Ramel S, Mely L, Hamidfar R, Douvry B, Houdouin V, Audousset C, Macey J, Mittaine M, Weiss L, Cosson L, Danner-Boucher I, Reix P, Baravalle M, Burnet E, Sermet-Gaudelus I, Martin C, Paillasseur JL, French Cystic Fibrosis Reference Network study group

doi: 10.1016/S2213-2600(25)00190-0

RECOMMENDED

Pulmonary FibrosisBronchi

Effect of elexacaftor-tezacaftor-ivacaftor on bronchial dilatations in adolescents with cystic fibrosis: a multicentre prospective observational study

★ 3 Recommended by Dr Martin Frey, Faculty Member

Lancet Respir Med

Sermet-Gaudelus I, Letierce A, Berteloot L, Bonnel AS, Chen Y, Makani P, Kelly-Aubert M, Kanoun F, Penalva L, Bouleghem N, Bihouee T, Bui S, Corvol H, Houdouin V, Mittaine M, Tatopoulos A, Weiss L, Wizla N, Kapel N, Bessou A, Tiddens HAWM, Caudri D, Reix P, Marguet C, MODUL-CF study group

doi: 10.1016/S2213-2600(25)00248-6

Pulmonary FibrosisFurther Publications

Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study

Ther Adv Respir Dis

Pepe A, Fevola C, Dolce D, Campana S, Ravenni N, Taccetti G, Salvatore D, Terlizzi V

doi: 10.1177/17534666251314706

RECOMMENDED

Pulmonary Fibrosis

Severe hepatotoxicity is uncommon following the introduction of Elexacaftor/Tezacaftor/Ivacaftor: A real-world two-years follow-up study of the Danish cystic fibrosis cohort

★ 1 Recommended by Dr Martin Frey, Faculty Member

J Cyst Fibros

Højte C, Olsen MF, Faurholt-Jepsen D, Jørgensen MH, Pressler T, Leo-Hansen C, Bryrup T, Jeppesen M, Olesen HV, Petersen J, Katzenstein TL, Nielsen BU, Skov M, TransformCF Study Group

doi: 10.1016/j.jcf.2025.08.008

Pulmonary Fibrosis

Improvements in health-related quality of life in people with cystic fibrosis ≥6 years of age treated with vanzacaftor/tezacaftor/deutivacaftor

J Cyst Fibros

Wainwright CE, Thorat T, Conner S, Quon BS, Duckers J, Lou Y, Mo C, Xue Y, Zhu L, McGarry L

doi: 10.1016/j.jcf.2025.11.014

RECOMMENDED

Pulmonary Fibrosis

Elexacaftor/tezacaftor/ivacaftor is associated with long-term reduction in use of chronic respiratory therapies in cystic fibrosis

★ 2 Recommended by Dr Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

J Cyst Fibros

Sagel SD, Vu P, Heltshe SL, Solomon GM, Kelly A, Pittman J, Rosenfeld M, Ratjen F

doi: 10.1016/j.jcf.2025.11.016

RECOMMENDED

Further Publications

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Glucose Tolerance and Abnormal Glucose Metabolism: A Phase 3b, Open-Label Clinical Trial

★ 2 Recommended by Dr Martin Frey, Faculty Member

Am J Respir Crit Care Med

Durieu I, Clements B, Fabrizzi B, Mall MA, McKone E, Ramsey B, Tullis E, Taylor-Cousar JL, van der Meer R, Bachman E, Chin A, Conner S, Jennings M, Weinstock T, Colombo C, Robinson P

doi: 10.1164/rccm.202411-2312OC

Asthma

Assessment of the role of small airway dysfunction in relation to exacerbation risk in patients with well controlled asthma (ATLANTIS): an observational study

Lancet Respir Med

Galant SP, Kuks PJM, Kole TM, Kraft M, Siddiqui S, Fabbri LM, Beghé B, Rabe KF, Papi A, Brightling CE, Singh D, Kocks JWH, Franzini L, Vonk JM, Kerstjens HAM, Heijink IH, Pouwels SD, Slebos DJ, van den Berge M

doi: 10.1016/S2213-2600(25)00283-8

Related content

LONGITUDE: An observational study of the long-term effectiveness of elexacaftor/tezacaftor/ivacaftor in people aged ≥12 years with cystic fibrosis using data from the United Kingdom Cystic Fibrosis Registry - 2-year analysis

Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies

Effect of elexacaftor-tezacaftor-ivacaftor on bronchial dilatations in adolescents with cystic fibrosis: a multicentre prospective observational study

Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study

Severe hepatotoxicity is uncommon following the introduction of Elexacaftor/Tezacaftor/Ivacaftor: A real-world two-years follow-up study of the Danish cystic fibrosis cohort

Improvements in health-related quality of life in people with cystic fibrosis ≥6 years of age treated with vanzacaftor/tezacaftor/deutivacaftor

Elexacaftor/tezacaftor/ivacaftor is associated with long-term reduction in use of chronic respiratory therapies in cystic fibrosis

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Glucose Tolerance and Abnormal Glucose Metabolism: A Phase 3b, Open-Label Clinical Trial

Assessment of the role of small airway dysfunction in relation to exacerbation risk in patients with well controlled asthma (ATLANTIS): an observational study