Airway Imaging Practices in Primary Ciliary Dyskinesia: A Global Survey to Guide Standardized Guidelines

Airway imaging guidelines for Primary Ciliary Dyskinesia (PCD) are currently limited. Given the progressive nature of this disease, standardized imaging protocols, particularly at key clinical milestones such as diagnosis, routine reviews, and exacerbations, may improve monitoring of disease progression, support comparison of outcomes across centres, and provide robust parameters for clinical trials.

To document current practices, we conducted a global survey of PCD clinicians. An online questionnaire was distributed via international PCD networks, covering imaging use at diagnosis, during routine assessments, and at exacerbations treated with oral or intravenous antibiotics. Responses were received from 120 clinicians across 90 centres in 33 countries. Modalities assessed included X-ray, CT scan, MRI, and ultrasound, with analyses stratified by centre type and size.

At diagnosis, 94% of clinicians reported using imaging. Chest X-ray was most common for children under 5 years (55%), whereas CT was preferred for older patients (66%). During routine assessments, 67.5% of clinicians used imaging, but timing and modality varied, with CT more frequent in paediatric and mixed centres. Imaging during exacerbations differed by treatment route: chest X-ray was the most common modality, used by 57.1% for orally antibiotic-treated episodes and 73.3% for intravenous episodes. CT use was higher with intravenous treatment (15.8% vs. 4.2%).

Although practices varied, consistent trends at diagnosis and follow-up highlight opportunities for consensus.

Standardized imaging protocols could enhance clinical care, enable meaningful research comparisons, and improve outcomes in PCD.