Magnetic resonance imaging detects dilated bronchial arteries in patients with primary ciliary dyskinesia
Author Block: D. Cetiner1, P. Leutz-Schmidt1, M. Stahl2, O. Sommerburg1, C. P. Heußel1, H-U. Kauczor1, M. A. Mall2, M. O. Wielpütz3, L. Wucherpfennig1; 1Heidelberg/DE, 2Berlin/DE, 3Greifswald/DE
Purpose: Bronchial arterial dilatation (BAD) is known to be associated with pulmonary hemorrhage and is highly prevalent in patients with chronic muco-obstructive lung diseases such as cystic fibrosis. However, studies on the prevalence of BAD in patients with primary ciliary dyskinesia (PCD) and their association with lung disease severity are lacking. Thus, the aim of our study is to evaluate BAD onset and its association with disease severity in patients from two different centers with PCD by magnetic resonance imaging (MRI).
Methods or Background: 68 patients with PCD (mean age at baseline 17.3±14.4 yr) performed annual standardized MRI including dynamic contrast-enhanced (DCE) MRI or angiography, contributing to a total of 155 examinations. The presence of BAD was assessed visually by two readers in consensus. Disease severity was assessed using the validated chest MRI score and spirometry (percent predicted forced expiratory volume in 1s [ppFEV1]).
Results or Findings: At baseline, at least one BAD was present in 30 patients (44.1%), with most patients having one or two BAD (53.3% and 26.6%, respectively). The number of BAD correlated strongly with patient age (r=0.641, p<0.001). The mean MRI global score was significantly higher in patients with BAD at baseline compared to patients without BAD (26.3±6.7 vs. 19±8.1, p=0.002), while ppFEV1 did not show differences (76.2±20.8% vs. 73.1±18.5%, p=0.57). During follow-up, four patients developed a BAD for the first time (5.8%) and three patients (4.4%) developed an additional BAD. BAD development was neither associated with changes in the MRI score, nor in ppFEV1 (p>0.99 and p=0.87).
Conclusion: Our study demonstrates a high prevalence of BAD in children and adults with PCD and an association between prevalence of BAD markers of greater disease severity.
Limitations: Not all patients received angiography additional to DCE-MRI.
Funding for this study: This study was supported by grants from the German Federal Ministry of Education and Research (BMBF) (82DZL004A1, 82DZL009C1 and 01GL2401A) and the German Research Foundation (CRC 1449 – project 431232613). MS is participant of the BIH-Charité Clinician Scientist Program funded by the Charité – Universitätsmedizin Berlin and the BIH. LW is participant of the Career Development Programm funded by the University of Heidelberg. Funders had no involvement in the collection, analysis and interpretation of data, in the writing of the report and in the decision to submit the article for publication.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Ethic commitee approval number: S-509/2019, S-184/2025, EA2/003/21
University Hospital Heidelberg