Interrater Reliability of a Modified Bronchoscopy Scoring Tool in Children With Cystic Fibrosis
Open Access
- 30 December 2025
- journal article
- Published by Wiley in Pediatric Pulmonology
- Vol. 61 (1) , e71452
Abstract
Background: Flexible bronchoscopy (FB) is widely used in the management of children with Cystic Fibrosis (CF) to visualize airway abnormalities, assess inflammation and detect infection. While previous scoring systems have been proposed to quantify visual airway findings in general pediatric populations, no standardized tool exists for assessing airway inflammation specific to CF. Methods: We modified the previously proposed pediatric bronchoscopy scoring tool by adding four features relevant to CF pathology: mucus plugging, secretion viscosity, bleeding, and vascular drawing (abnormal or enhanced visualization of airway mucosal vessels, reflecting neovascular remodeling associated with inflammation). Eighty bronchoscopy recordings (50 CF, 30 non‐CF) were retrospectively scored by four raters blinded to the clinical information, and ten visual features were assessed: six from the previously proposed score (secretion amount and color, mucosal edema, erythema, ridging, and pallor) and the four CF‐specific additions. Inter‐rater reliability was assessed using Gwet's AC2 coefficient. Results: Agreement between raters varied across features. Mucus plugging (AC2 = 0.94) and bleeding (0.80), both CF‐specific, were among the most reliably scored features, while secretion viscosity (0.47) and vascular drawing (0.50) showed the lowest agreement. The expanded score demonstrated comparable or improved reliability for overlapping features from earlier scoring systems. Conclusion: The modified bronchoscopy score demonstrated moderate to excellent inter‐rater reliability and added clinically relevant features specific to CF. It may serve as a standardized method to assess bronchoscopy for pediatric CF lung disease, although further validation is needed for features with lower inter‐rater reliability.Keywords
This publication has 20 references indexed in Scilit:
- A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trialThe Lancet Respiratory Medicine, 2014
- Bronchoscopic Findings in Children With Chronic Wet CoughPediatrics, 2012
- A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutationNew England Journal of Medicine, 2011
- Infection, Inflammation, and Lung Function Decline in Infants with Cystic FibrosisAmerican Journal of Respiratory and Critical Care Medicine, 2011
- Sputum colour: a useful clinical tool in non-cystic fibrosis bronchiectasisEuropean Respiratory Journal, 2009
- Computing inter‐rater reliability and its variance in the presence of high agreementBritish Journal of Mathematical and Statistical Psychology, 2008
- Massive Hemoptysis in Cystic FibrosisChest, 2005
- Angiogenesis and Remodeling of Airway Vasculature in Chronic InflammationAmerican Journal of Respiratory and Critical Care Medicine, 2001
- The Bronchitis IndexChest, 1993
- The Measurement of Observer Agreement for Categorical DataBiometrics, 1977