Research graph
References from Residual disease activity in cystic fibrosis lung disease: implications for clinical management and research. Local targets link to admitted publications; unresolved targets remain external evidence.
Cystic fibrosis
10.1038/s41572-024-00538-6 · 2024 · External reference
Triple therapy for cystic fibrosis Phe508del-gating and -residual function genotypes
10.1056/nejmoa2100665 · 2021 · External reference
Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials
10.1016/s2213-2600(24)00411-9 · 2025 · External reference
The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study
10.1016/s2213-2600(24)00208-x · 2024 · External reference
Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele
10.1056/nejmoa1908639 · 2019 · External reference
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial
10.1016/s0140-6736(19)32597-8 · 2019 · External reference
Clinical effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: a clinical trial
10.1164/rccm.202108-1986oc · 2022 · External reference
Effects of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in patients with cystic fibrosis and one or two f508del alleles
10.1164/rccm.202201-0219oc · 2022 · External reference
Improvement in lung clearance index and chest computed tomography scores with elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis aged 12 years and older—the RECOVER trial
10.1164/rccm.202308-1317oc · 2023 · External reference
Rapid improvement after starting elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis and advanced pulmonary disease
10.1164/rccm.202011-4153oc · 2021 · External reference
Advances in cystic fibrosis: CFTR modulator triple combinations
10.1183/13993003.01783-2025 · 2026 · External reference
Elexacaftor/tezacaftor/ivacaftor for cystic fibrosis and rare CFTR variants: in vitro translation to a phase 3, double-blind, randomized, placebo-controlled trial and real-world study
10.1093/ajrccm/aamaf001 · 2026 · External reference
Elexacaftor/tezacaftor/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del
10.1016/j.jcf.2023.02.004 · 2023 · External reference
The ageing of people living with cystic fibrosis: what to expect now?
10.1183/16000617.0071-2024 · 2024 · External reference
Efficacy and safety of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis heterozygous for F508del and a minimal function mutation: a phase 3b, randomized, placebo-controlled study
10.1164/rccm.202202-0392oc · 2022 · External reference
Elexacaftor/tezacaftor/ivacaftor in children aged ≥6 years with cystic fibrosis heterozygous for F508del and a minimal function mutation: results from a 96-week open-label extension study
10.1183/13993003.02435-2024 · 2025 · External reference
Phase 3 open-label clinical trial of elexacaftor/tezacaftor/ivacaftor in children aged 2-5 years with cystic fibrosis and at least one F508del allele
10.1164/rccm.202301-0084oc · 2023 · External reference
Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two F508del alleles
10.1183/13993003.00004-2024 · 2024 · External reference
Is disease stability an attainable chronic obstructive pulmonary disease treatment goal?
10.1164/rccm.202406-1254ci · 2025 · External reference
2025 American College of Rheumatology (ACR) guideline for the treatment of systemic lupus erythematosus
10.1002/acr.25690 · 2026 · External reference
2019 update of the American College of Rheumatology recommended rheumatoid arthritis disease activity measures
10.1002/acr.24042 · 2019 · External reference
Disease severity and activity in bronchiectasis: a paradigm shift in bronchiectasis management
10.4046/trd.2024.0120 · 2025 · External reference
Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry
10.1183/09031936.00166412 · 2014 · External reference
Hallmarks of health
10.1016/j.cell.2020.11.034 · 2021 · External reference
Heterogeneity in response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis
10.1016/j.jcf.2024.04.013 · 2024 · External reference
Heterogeneity of CFTR modulator-induced sweat chloride concentrations in people with cystic fibrosis
10.1016/j.jcf.2024.02.001 · 2024 · External reference
Variability in disease severity among cystic fibrosis patients carrying residual-function variants: data from the European Cystic Fibrosis Society Patient Registry
10.1183/23120541.00587-2024 · 2025 · External reference
Sweat chloride reflects CFTR function and correlates with clinical outcomes following CFTR modulator treatment
10.1016/j.jcf.2024.12.006 · 2025 · External reference
Effects of elexacaftor/tezacaftor/ivacaftor therapy on CFTR function in patients with cystic fibrosis and one or two F508del alleles
10.1164/rccm.202110-2249oc · 2022 · External reference
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials
10.1164/rccm.200509-1377oc · 2006 · External reference
CFTR Cl-channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis
10.1053/j.gastro.2004.07.006 · 2004 · External reference
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis
10.1183/13993003.02153-2022 · 2023 · External reference
Standards for the care of people with cystic fibrosis; establishing and maintaining health
10.1016/j.jcf.2023.12.002 · 2024 · External reference
Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy
10.1183/13993003.00616-2025 · 2026 · External reference
Effect of elexacaftor/tezacaftor/ivacaftor on mucus and mucociliary clearance in cystic fibrosis
10.1016/j.jcf.2023.10.010 · 2024 · External reference
Discontinuation of supportive therapies in people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor—a patient-reported outcomes’ study
10.1016/j.rmed.2025.108464 · 2025 · External reference
Relationship between lung volumes and heterogeneity in the response to elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease
10.1016/j.chest.2024.03.033 · 2024 · External reference
Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor
10.1183/13993003.01794-2023 · 2024 · External reference
Effect of elexacaftor-tezacaftor-ivacaftor on bronchial dilatations in adolescents with cystic fibrosis: a multicentre prospective observational study
10.1016/s2213-2600(25)00248-6 · 2026 · External reference
Elexacaftor/tezacaftor/ivacaftor’s effects on cystic fibrosis infections are maintained, but not increased, after 3.5 years of treatment
10.1172/jci184171 · 2024 · External reference
Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy
10.1183/16000617.0068-2024 · 2024 · External reference
Changes in microbiome dominance are associated with declining lung function and fluctuating inflammation in people with cystic fibrosis
10.3389/fmicb.2022.885822 · 2022 · External reference
Impact of CFTR modulation on Pseudomonas aeruginosa infection in people with cystic fibrosis
10.1093/infdis/jiae051 · 2024 · External reference
Sputum metagenomics in bronchiectasis reveals pan-European variation: an EMBARC-BRIDGE study
10.1183/13993003.00054-2025 · 2025 · External reference
Characteristics, treatment and lung function outcomes of pulmonary exacerbations in cystic fibrosis: insights from the BEAT-CF cohort
10.1183/13993003.01349-2025 · 2026 · External reference
Differences in pulmonary exacerbation risks among CF subpopulations
10.1016/j.jcf.2025.11.010 · 2026 · External reference
Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis
10.1183/13993003.00150-2025 · 2025 · External reference
Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis
10.1136/thorax-2022-219943 · 2023 · External reference
Pharmacological improvement of cystic fibrosis transmembrane conductance regulator function rescues airway epithelial homeostasis and host defense in children with cystic fibrosis
10.1164/rccm.202310-1836oc · 2024 · External reference
Transcriptomic and functional responses of the cystic fibrosis airway epithelium to CFTR modulator therapy
10.1172/jci.insight.196018 · 2025 · External reference
Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist
10.1172/jci167957 · 2023 · External reference
Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections
10.1164/rccm.201609-1954oc · 2017 · External reference
Sputum colour: a useful clinical tool in non-cystic fibrosis bronchiectasis
10.1183/09031936.00163208 · 2009 · External reference
The sputum colour chart as a predictor of lung inflammation, proteolysis and damage in non-cystic fibrosis bronchiectasis: a case-control analysis
10.1111/resp.12219 · 2014 · External reference
Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)
10.1183/13993003.01554-2023 · 2024 · External reference
Improvements in health-related quality of life in people with cystic fibrosis ≥6 years of age treated with vanzacaftor/tezacaftor/deutivacaftor
10.1016/j.jcf.2025.11.014 · 2026 · External reference
An amazing transformation!” The lived experiences of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis
10.1159/000549098 · 2026 · External reference
Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adults and adolescents with cystic fibrosis and at least one F508del allele: a phase 3 open-label extension study
10.1164/rccm.202411-2231oc · 2025 · External reference
Symptoms and quality of life in adults with cystic fibrosis: a cross-sectional analysis of the InSPIRe: CF trial
10.1016/j.jcf.2024.03.005 · 2024 · External reference
A refresh of the top 10 research priorities in cystic fibrosis
10.1136/thorax-2023-220100 · 2023 · External reference
Time out for an hour, every day, my whole life”: understanding treatment burden in cystic fibrosis in the era of elexacaftor/tezacaftor/ivacaftor (ETI)
10.1016/j.jcf.2025.07.014 · 2025 · External reference
Baseline characteristics and self-reported use of chronic daily therapies of the home reported outcomes with CFTR modulator therapy (HERO-2) cohort
10.1002/ppul.71190 · 2025 · External reference
Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials
10.1016/s2213-2600(22)00434-9 · 2023 · External reference
The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches
10.1016/s0140-6736(23)01608-2 · 2023 · External reference
A phase IIa, single-blind, placebo-controlled, parallel-group study to assess safety, tolerability, and pharmacokinetics/pharmacodynamics of brensocatib in adults with cystic fibrosis
10.1007/s40262-025-01550-z · 2025 · External reference
Cathepsin C (dipeptidyl peptidase 1) inhibition in adults with bronchiectasis: AIRLEAF, a phase II randomised, double-blind, placebo-controlled, dose-finding study
10.1183/13993003.01551-2024 · 2025 · External reference
Neutrophil serine proteases in cystic fibrosis: role in disease pathogenesis and rationale as a therapeutic target
10.1183/16000617.0001-2024 · 2024 · External reference
Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE
10.1002/art.1780350606 · 1992 · External reference
Judging disease activity in clinical practice in rheumatoid arthritis: first step in the development of a disease activity score
10.1136/ard.49.11.916 · 1990 · External reference
Pulmonary endpoints in clinical trials for children with cystic fibrosis under two years of age
10.1016/j.jcf.2025.06.003 · 2025 · External reference
