Real-World Impact of Elexacaftor/Tezacaftor/Ivacaftor on Health-Related Quality of Life in Adults with Cystic Fibrosis: A 12-Month Multicenter Cohort Study

Pulmonary Fibrosis

Real-World Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis: A 24-Month Italian National Registry Study

J Clin Med

Salvatore D, Campagna G, Padoan R, Pepe A, Amato A, Salvatore M

doi: 10.3390/jcm15072699

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. med. 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

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 FibrosisRespiratory Tract Infection

Real -World impact of previous exposure to CFTR modulators on clinical parameters and chronic sinus disease in people with cystic fibrosis on elexacaftor-tezacaftor-ivacaftor

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Respir Med

Holtzlander M, Lind M, Eisner M, Gushue C, Palacios S, Kotha K, Imran S, McCoy K, Sheikh S

doi: 10.1016/j.rmed.2025.108274

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

Pulmonary HypertensionFurther Publications

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

Ther Adv Respir Dis

Blankenship S, Flueckiger BV, Caleb G, Bailey J, Baker E, Garcia B, Krick S

doi: 10.1177/17534666251410246

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 FibrosisBronchi

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

★ 3 Recommended by Dr. med. 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

RECOMMENDED

Pulmonary Fibrosis

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adolescents and adults with cystic fibrosis and F508del-gating and F508del-residual function genotypes: Results from an open-label extension study

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Ann Am Thorac Soc

Chmiel JF, McBennett K, Quon BS, Colombo C, De Wachter E, Fajac I, Mondejar-Lopez P, Polineni D, Robinson P, Sutharsan S, Mall MA, McKone EF, Ramsey B, Taylor-Cousar JL, Tullis E, Suresh NE, Zhang Y, Sosnay P, Jennings M, Barry PJ, 445-110 study group

doi: 10.1093/annalsats/aaoag053

Related content

Real-World Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis: A 24-Month Italian National Registry 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

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

Real -World impact of previous exposure to CFTR modulators on clinical parameters and chronic sinus disease in people with cystic fibrosis on elexacaftor-tezacaftor-ivacaftor

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

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

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

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

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adolescents and adults with cystic fibrosis and F508del-gating and F508del-residual function genotypes: Results from an open-label extension study