Cystic fibrosis year in review 2025

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 Fibrosis

Medication adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: Analysis on the French cystic fibrosis population from 2012-2020

Respir Med

Eid E, Payet C, Fernandez V, Belhassen M, Bessou A, Durieu I, Reynaud Q, Viprey M, French Cystic Fibrosis Centers Network, Cinthia R, Françoise T, Marie-Laure D, Bénédicte RT, Stéphanie B, Julie M, Murie L, Sylvie M, Ralph E, Frédéric H, Manuela S, Laurent M, Rébecca H, Catherine L, Camille A, Nathalie WD, Jane L, Philippe R, Jean-Christophe D, Martine RG, Raphaël C, Aurélie T, Anne G, Isabelle DB, Tiphaine B, Sylvie L, Pierre-Régis B, Isabelle S, Véronique H, Harriet C, Katia BK, Chantal B, Eric D, Sophie R, Christophe M, Nathalie A, Caroline P, Benjamin RP, Laurence W, Dominique G, Léa R, Marlène ME, Julie M, Laure C, Baptiste A, Gabsi Asma

doi: 10.1016/j.rmed.2026.108857

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

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

RECOMMENDED

Pulmonary FibrosisBronchi

Efficacy of Anti-Inflammatory Therapies for Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and Network Meta-Analysis

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Chest

Yamamoto S, Niitsu T, Fukushima K, Shiozawa A, Imai R, Hashimoto K, Olivier KN, Aksamit TR, Morimoto K

doi: 10.1016/j.chest.2025.12.035

RECOMMENDED

Pulmonary Fibrosis

The diagnostic challenge of pancreatic cysts in patients with cystic fibrosis: a case series and review of the literature

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

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

J Cyst Fibros

Fleurent A, Miseur B, Weynand B, Topal H, Dupont L, van Malenstein H

doi: 10.1016/j.jcf.2026.02.007

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 FibrosisFurther Publications

Clinical efficacy of elexacaftor-tezacaftor-ivacaftor in two siblings with homozygous I1234V mutation cystic fibrosis: A prospective case series

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

Respir Med Case Rep

Alzaid M, Alshahrani T, Alotaibi R, Mukhtar G, Alanazi A, Alsadoon H, Eltahir S, Aldraihem A, Alotaibi W

doi: 10.1016/j.rmcr.2025.102264

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. med. 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

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

Medication adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: Analysis on the French cystic fibrosis population from 2012-2020

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

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

Efficacy of Anti-Inflammatory Therapies for Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and Network Meta-Analysis

The diagnostic challenge of pancreatic cysts in patients with cystic fibrosis: a case series and review of the literature

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

Clinical efficacy of elexacaftor-tezacaftor-ivacaftor in two siblings with homozygous I1234V mutation cystic fibrosis: A prospective case series

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