Upper airway disease in PCD: Clinical management and factors influencing decision-making

Background Upper airway disease is common in primary ciliary dyskinesia (PCD), but management evidence is limited. We aimed to describe management practices and identify factors influencing management decisions. Methods Using data from the Ear-Nose-Throat (ENT) Prospective International Cohort of patients with PCD (EPIC-PCD) and an ENT-specialist survey across participating centres, we described management practices recorded at routine follow-up. We assessed clinical factors associated with practices via mixed-effects logistic regression models. In a subgroup of patients, we assessed factors associated with initiation or discontinuation of practices. Results We included 579 patients: median age 15 years, 46% female. Nasal rinsing (54%) and nasal corticosteroids (22%) were most frequently prescribed. Among 466 patients with available data, 47 had grommets (10%) and 42 hearing aids (9%). Nasal corticosteroids and rinsing were more frequently prescribed in patients with polyps (odds ratio [OR] 3.74, 95% confidence interval [CI] 1.80–7.76; OR 3.39, 95% CI 1.37–8.37) or turbinate hypertrophy (OR 1.89, 95% CI 1.03–3.47; OR 2.89, 95% CI 1.55–5.38), and upper airway nebulisation in patients with frequent nasal symptoms (OR 2.86, 95% CI 1.11–7.39). Management practices differed between centres, as seen also by the specialists’ survey responses. In 177 patients with multiple visits, initiation of nasal rinsing was associated with frequent nasal symptoms (OR 3.18, 95% CI 1.24–8.18) and turbinate hypertrophy (OR 3.21, 95% CI 1.20–8.59). Conclusion Upper airway disease management in PCD varies and is partly guided by symptom burden and clinical findings. This variation across centres highlights the need for care standardisation and PCD-specific management guidelines.

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Journal
ERJ Open Research
Published
2026-09-24
DOI
https://doi.org/10.1183/23120541.00866-2026
Primary Topic
Cystic Fibrosis Research Advances
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article
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article

Upper airway disease in PCD: Clinical management and factors influencing decision-making

Tuğba Şişmanlar Eyüboğlu, Mihaela Alexandru, Oğuzhan Katar, Isolde Hayn et al.
ERJ Open Research
Cystic Fibrosis Research Advances
article

Upper airway disease in PCD: Clinical management and factors influencing decision-making

Tuğba Şişmanlar Eyüboğlu, Mihaela Alexandru, Oğuzhan Katar, Isolde Hayn, E Ertem Eralp, Céline Kempeneers, Andreas Anagiotos, Stephanie A. Thee, Mieke Boon, Nagehan Emiralioğlu, Panayiotis Yiallouros, Nena Karavasiloglou, Charlotte O. Pioch, Myrofora Goutaki, Dafni Moriki, Yasemi̇n Gökdemi̇r, Rico N. P. M. Rinkel, Anne‐Lise Poirrier, Suzanne Crowley, Ana Reula, Bulent Taner Karadag, Hasnaa Ismail-Koch, Jean‐François Papon, M Armengot, Johanna Raidt, Uğur Özçelik, Ayşe Tana Aslan, Andrea Fernandez-Rodriguez, Vasiliki Gkatzou, Eric G. Haarman, Nathalie Isabelle Caversaccio, Eleonore de Garempel de Bressieux, Alexis Campos, Amanda Harris, on behalf of the EPIC-PCD team, Sinan Ahmed D. Dheyauldeen, I. C. M. Bon
article en

Abstract

Background Upper airway disease is common in primary ciliary dyskinesia (PCD), but management evidence is limited. We aimed to describe management practices and identify factors influencing management decisions. Methods Using data from the Ear-Nose-Throat (ENT) Prospective International Cohort of patients with PCD (EPIC-PCD) and an ENT-specialist survey across participating centres, we described management practices recorded at routine follow-up. We assessed clinical factors associated with practices via mixed-effects logistic regression models. In a subgroup of patients, we assessed factors associated with initiation or discontinuation of practices. Results We included 579 patients: median age 15 years, 46% female. Nasal rinsing (54%) and nasal corticosteroids (22%) were most frequently prescribed. Among 466 patients with available data, 47 had grommets (10%) and 42 hearing aids (9%). Nasal corticosteroids and rinsing were more frequently prescribed in patients with polyps (odds ratio [OR] 3.74, 95% confidence interval [CI] 1.80–7.76; OR 3.39, 95% CI 1.37–8.37) or turbinate hypertrophy (OR 1.89, 95% CI 1.03–3.47; OR 2.89, 95% CI 1.55–5.38), and upper airway nebulisation in patients with frequent nasal symptoms (OR 2.86, 95% CI 1.11–7.39). Management practices differed between centres, as seen also by the specialists’ survey responses. In 177 patients with multiple visits, initiation of nasal rinsing was associated with frequent nasal symptoms (OR 3.18, 95% CI 1.24–8.18) and turbinate hypertrophy (OR 3.21, 95% CI 1.20–8.59). Conclusion Upper airway disease management in PCD varies and is partly guided by symptom burden and clinical findings. This variation across centres highlights the need for care standardisation and PCD-specific management guidelines.

ERJ Open Research
Oslo University Hospital (NO), Ankara University (TR), University of Liège (BE), National and Kapodistrian University of Athens (GR), Université Paris-Saclay (FR), Nicosia General Hospital (CY), Universidad Cardenal Herrera CEU (ES), University Hospital of Bern (CH), Hospital Universitari i Politècnic La Fe (ES), University of Cyprus (CY), Emma Kinderziekenhuis (NL), Assistance Publique – Hôpitaux de Paris (FR), University Hospital Münster (DE), Southampton Children's Hospital (GB), Institute of Social and Preventive Medicine (CH), University General Hospital Attikon (GR), Amsterdam University Medical Centers (NL), Bicêtre Hospital (FR), Hacettepe University (TR), Marmara University (TR), Charité - Universitätsmedizin Berlin (DE), Vrije Universiteit Amsterdam (NL), University of Amsterdam (NL), Gazi University (TR), KU Leuven (BE)
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