Sinonasal disease among patients with primary ciliary dyskinesia – an international study

Background Although most patients with primary ciliary dyskinesia (PCD) report sinonasal symptoms, little is known about symptom frequency and severity. We describe sinonasal manifestations among PCD patients using data from the Ear, nose, and throat (ENT) Prospective International Cohort of PCD patients. Methods We included data from participants with routine clinical ENT examinations and complete FOLLOW-PCD symptoms questionnaires from the same visit or within 2 weeks. We compared the prevalence, reported symptoms, and clinical findings among children and adults and identified potential factors associated with increased risk of sinonasal disease using ordinal regression. Results We included 397 (53% male) participants from 12 centres with median age 15 years (IQR 9– 22). Almost all (352; 89%) reported chronic nasal symptoms. More adults (34; 26%) than children (11; 5%) reported anosmia or hyposmia. Among 140 participants who completed SNOT-22 questionnaires, quality of life was moderately affected by sinonasal symptoms (median score 31; IQR 22–45). We observed nasal polyps among 52 (15%) of 352 participants and hypertrophic inferior nasal turbinates among 129 (34%) of 353 participants; facial pain was recorded among 51 (13%) of 353 participants. More adults than children had nasal polyps, hypertrophic turbinates, deviated septum, and facial pain. We found age 10 and older the only factor associated with increased risk of sinonasal disease. Conclusions Our study reinforces the importance of regular sinonasal evaluations for patients of all ages with PCD and the need for developing evidence-based guidelines for sinonasal treatments as part of overall PCD management. ### Competing Interest Statement P Latzin received grants or honoraria for participation in data safety monitoring boards or advisory boards from Vertex, Vifor, OM Pharma, Polyphor, Santhera (DMC), and Sanofi Aventis within the last 36 months. J Roehmel received grants, clinical study recompensations from Vertex, INSMED, Medical Research Council/UK, BMBF, Mukoviszidose Institut. ### Clinical Trial NCT04611516 ### Clinical Protocols ### Funding Statement This study is funded by a Swiss National Science Foundation Ambizione fellowship (PZ00P3_185923). ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: The details of the IRB/oversight body that provided approval or exemption for the research described are given below: The study has been reviewed and approved by the local Human Research Ethics Committees at every participating centre, based on local legislation. We list below the names of the ethics committees which approved the study and the approval reference numbers, when applicable. A. The following centres have a pre-existing or new ethical approval, which allows the contribution of pseudonymised data to observational collaborative international studies (covers the EPIC-PCD study): 1.University Childrens Hospital Charite-Universitaetsmedizin, Berlin, Germany: Ethical Committee Charite (EA2/003/21) 2. University Childrens hospital, Bern, Switzerland: Cantonal Ethics Committee of Bern (KEKBE: 060/2015) 3. University of Cyprus: Ethical Committee for biomedical research in Leukosia Cyprus (EEBK/EΠ/2013/21) 4. Marmara University Istanbul, Turkey: Ethical Committee of Marmara University (09.2018.395) 5. University Hospital of Southampton, United Kingdom: Southampton and South West Hampshire research ethics committee (06/Q1702/109) B. The following centres applied for ethical approval to participate specifically to the EPIC-PCD study: 1. VU University medical center (VUmc), Amsterdam, The Netherlands: The Medical Ethics Review Committee of VU University Medical Center reviewed the application and concluded on 24th of November 2020 that no approval is needed to participate to the EPIC-PCD cohort as the Medical Research Involving Human Subjects Act does not apply to the study. 2. Hacettepe University, Ankara, Turkey: Non-interventional clinical research EC of Hacettepe University (2020/11-47) 3. University Hospital of Leuven, Belgium: Ethical Committee for Research of University Hospitals Leuven (S64411) 4. Hospital Universitario La Fe in Valencia, Spain: Ethical Committee of medical investigations of Hospital Universitario La Fe (2020-498-1) 5. University Hospital Bicetre Paris-Sud, Paris, France: The AP-HP Direction de la Recherche Clinique et de l'Innovation reviewed the application and concluded on 4th of February 2021 that that no approval is needed to participate to the EPIC-PCD cohort as the Jarde law that regulates clinical research in France does not apply to the study. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines and uploaded the relevant EQUATOR Network research reporting checklist(s) and other pertinent material as supplementary files, if applicable. Yes The datasets used and analysed during the current study are available from the study PI Dr Myrofora Goutaki (myrofora.goutaki{at}ispm.unibe.ch) upon reasonable request.