Assessment of Structural Lung Disease in Cystic Fibrosis
Addressing current challenges
Cystic Fibrosis (CF) lung disease is defined by progressive structural airway abnormalities that begin in infancy. These changes often emerge before functional decline is detectable, persisting throughout a patient’s life despite therapeutic advances.
Although CT is considered as the gold standard for assessing structural lung disease in CF, current CT-based visual scoring methods struggle to provide the standardized and quantitative data required for longitudinal monitoring, particularly in large multi-center clinical trials. Traditional endpoints like FEV1 and exacerbation rates are too insensitive to structural changes to effectively track disease progression under CFTR modulators.
As advances in CF therapies shift the clinical focus toward early intervention and disease stabilization, there is a growing need for a more sensitive imaging metrics capable of capturing subtle structural changes to support effective and timely patient monitoring.
aged 18+
patient survival
Advancing treatment strategies with AI
With years of expertise and demonstrated sensitivity across the CF population, Thirona’s quantitative lung analysis captures critical structural changes in both the bronchial tree and lung parenchyma. Our technology enables precise, reproducible quantification of bronchial dilatation, airway wall thickening, mucus plug and trapped air.
Its clinical and scientific relevance is further strengthened through large-scale application in initiatives such as the ENRICH project, where standardized quantitative imaging supports consistent analysis across the European CF population and advances evidence generation in CF lung disease.
- Sensitive detection of early and subtle structural lung changes, including small-airway disease
- Reproducible longitudinal assessment of disease progression and treatment effects, including in pediatric populations
- Objective quantification of mucus burden and airway abnormalities, eliminating reader variability
- Standardized imaging biomarkers to support patient stratification and clinical trial endpoints
There is still much to uncover in Cystic Fibrosis and other rare lung diseases, where complex, patient-specific patterns of airway damage and progression define each patient’s journey. By extracting objective, patient-level metrics from CT imaging, AI-enabled quantitative analysis gives us the ability to tailor therapies to individual needs and response profiles.
Prof. Dr. Patrick Flume, MD
Medical University of South Carolina United States
Use cases advancing precision medicine
Longitudinal assessment of therapeutic response in Cystic Fibrosis
Major CF therapies aim at managing the disease and its symptoms. These include CFTR modulators that help correct issue with CFTR proteins and other therapies that reduce specific symptoms.
Multiple validation studies have utilized LungQ® measurements to evaluate the efficacy of ETI (elexacaftor/tezacaftor/ivacaftor), lum/iva (lumacaftor/ivacaftor), and inhaled hypertonic saline in pediatric populations. These studies demonstrate LungQ’s high sensitivity and accuracy in quantifying longitudinal disease progression and treatment response.
Validation studies
Journal of Cystic Fibrosis
| January 2026
Progression of structural lung disease and lung function in adolescents with cystic fibrosis
European Respiratory Journal
| January 2026
Structural lung abnormalities in children and adults with cystic fibrosis after ETI using AI automated algorithm
European Respiratory Journal
| January 2026
AI-based mucus plug analysis to evaluate the effect of inhaled hypertonic saline in preschool children with CF: a randomized controlled trial
European Respiratory Journal
| January 2026
The effect of Elexacaftor/Tezacaftor/Ivacaftor (ETI) on bronchial tapering as marker of bronchial dilatation in people with CF aged 12 above (RECOVER study)
Journal of Cystic Fibrosis
| May 2023
Automatic Bronchus and Artery Analysis on Chest Computed Tomography to Evaluate the Effect of Inhaled Hypertonic Saline in Children Aged 3-6 Years With Cystic Fibrosis in a Randomized Clinical Trial
American Journal of Respiratory and Critical Care Medicine
| May 2024
Fully Automatic Assessment of Bronchus-Artery Dimensions and Ratios, Mucus Plugs, and Low Attenuation Regions in Cystic Fibrosis Patients Before and After Elexacaftor-Tezacaftor-Ivacaftor
European Respiratory Journal (ERS Congress)
| September 2024
Lung vascular volumes in cystic fibrosis patients before and after Elexacaftor-Tezacaftor-Ivacaftor (ETI) therapy
Journal of Cystic Fibrosis
| July 2025
Improvements in structural lung disease in people with CF aged 12 and above on Elexacaftor/Tezacaftor/Ivacaftor (ETI) are sustained for up to two years
Journal of Cystic Fibrosis
| August 2025
Elexacaftor/tezacafor/ivacaftor improves bronchial dilatation in a real-world cohort of adolescents with cystic fibrosis
Monitoring of disease progression in Cystic Fibrosis
Cystic fibrosis (CF) lung disease is characterised by progressive bronchial wall thickening and widening. Traditionally, monitoring these changes requires radiologists to manually score CT scans. The recent research executed LungQ® BA analyses to measure bronchial artery dimension across a dataset of 23 manual annotated CT scans and two longitudinal datasets totaling 458CT scans in comparison with manual result.
It found that LungQ® BA analyses was shown to be sensitive in monitoring disease progression, demonstrating its equal ability to detect bronchial widening and wall thickening when compared to the manual “gold standard” scoring method.
Validation studies
Thorax
| September 2023
Automatic Analysis of Bronchus-Artery Dimensions to Diagnose and Monitor Airways Disease in Cystic Fibrosis
Journal of Cystic Fibrosis
| August 2025
Validation of an artificial intelligence-based automated PRAGMA and mucus plugging algorithm in pediatric cystic fibrosis
Inclusion of QCT metrics in CF population data
The European Cystic Fibrosis Society (ECFS) Patient Registry is introducing a standardized and validated method to quantify structural lung abnormalities from chest CT, enabling more accurate monitoring of therapeutic impact and disease progression in cystic fibrosis.
The registry is incorporating LungQ® analyses to provide precise quantification of structural lung abnormalities in its patient cohort (2500 patients and 5000 CT scans) and bring in critical, disease-relevant insights directly into the registry such as bronchial wall thickening, Mucus Plug Volume and Count, and air trapping.
