Quantitative CT of Lung Parenchyma and Airways in Primary Ciliary Dyskinesia
Author Block: O. Weinheimer1, S. Riepenhausen2, R. Tenardi-Wenge2, M. A. Mall3, H-U. Kauczor1, H. Omran2, M. O. Wielpütz4, J. Raidt2; 1Heidelberg/DE, 2Muenster/DE, 3Berlin/DE, 4Greifswald/DE
Purpose: Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder where tiny hair-like structures called cilia don’t work properly. Various quantitative CT (QCT) techniques have been proven to deliver useful and objective biomarkers describing lung parenchyma and airways. This study explores which QCT biomarkers best characterize lung and airway abnormalities in PCD.
Methods or Background: A total of 148 inspiratory CT scans from 106 genetically confirmed PCD subjects (55 female, mean age 29.2 ± 17.7 years), collected from multiple medical centers, were automatically analyzed using YACTA software. 59 CT scans from 35 individuals with situs inversus were mirrored to enable standard YACTA evaluation. Quantitative CT parameters of the airways and lung parenchyma were assessed on a six-lobe basis.
Results or Findings: Mirroring the CT scans allowed for the inclusion of all situs inversus subjects in the analysis. Airway wall percentage (AWP) was significantly higher in the lower lobes compared to the upper lobes (60.54% vs 55.89%, p<2.2e-16). Similarly, the bronchiectasis index (BEI) was elevated in the lower lobes (1.64 vs 0.45, p<2.2e-16). Mean lung density (MLD) and the 75th percentile of the lung histogram were also higher in the lower lobes (–729.39 HU vs –763.45 HU, p=1.95e-11 and –854.42 HU vs –892.74 HU, p=3.79e-10). The values in the right middle lobe and lingula were comparable to those in the lower lobes. AWP, BEI and MLD showed a moderate correlation with subject age (R=-0.57, p=8.91e-14 and R=0.27, p<0.001 and R=-0.47, p=2.85e-09).
Conclusion: QCT showed significant changes in airway and lung parenchyma paramaters predominantly in the lower lobes, differing from the typical pattern seen in cystic fibrosis, where changes are distributed across all lobes. QCT may be a valuable tool for PCD research.
Limitations: Retrospective study, no uniform CT protocol.
Funding for this study: DFG OM6/7, OM6/8, OM6/10, OM6/14, OM6/16, CRU 326 (subprojects OM6/11 (H. Omran), RA3522/1 (J. Raidt)) IZKF Muenster
(Om2/010/20; OM2/014/24), BMFTR - project ReproTrackMS (grant 01GR2303). Part of the authors are Healthcare Professionals in the European Reference Network ERN LUNG.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: The study was approved by the Ethics Committee of the Medical Association of Westphalia-Lippe and the local ethics committee of the University of Münster (Münster, Germany; AZ 2011-270-f-S).