Sinonasal disease among patients with primary ciliary dyskinesia: an international study
Authors: Lam Yin Ting , Papon Jean-François , Alexandru Mihaela , Anagiotos Andreas , Armengot Miguel , Boon Mieke , Burgess Andrea , Crowley Suzanne , Dheyauldeen Sinan Ahmed D. , Emiralioglu Nagehan , Erdem Eralp Ela , van Gogh Christine , Gokdemir Yasemin , Gunaydın Onder , Haarman Eric G. , Harris Amanda , Hayn Isolde , Ismail-Koch Hasnaa , Karadag Bülent , Kempeneers Céline , Kim Sookyung , Latzin Philipp , Lorent Natalie , Ozcelik Ugur , Pioch Charlotte , Poirrier Anne-Lise M.L. , Reula Ana , Roehmel Jobst , Yiallouros Panayiotis ,   on behalf of the EPIC-PCD team, Goutaki Myrofora ,   EPIC-PCD team:, Ademhan Dilber , Alexandru Mihaela , Anagiotos Andreas , Armengot Miguel , Benchimol Lionel , Beule Achim G. , Bon Irma , Boon Mieke , Bullo Marina , Burgess Andrea , Calmes Doriane , Casaulta Carmen , Caversaccio Marco , Caversaccio Nathalie , Crestani Bruno , Crowley Suzanne , Dheyauldeen Sinan Ahmed D. , Diepenhorst Sandra , Emiralioglu Nagehan , Eralp Ela Erdem , Ergenekon Pinar , Feyaerts Nathalie , Georgiou Gavriel , Glen Amy , Gogh Christine van , Gokdemir Yasemin , Goutaki Myrofora , Gunaydın Onder , Haarman Eric G. , Harris Amanda , Hayn Isolde , Helms Simone , Hool Sara-Lynn , Honoré Isabelle , Ismail Koch Hasnaa , Karadag Bülent , Kempeneers Céline , Kennelly Synne , Kieninger Elisabeth , Kim Sookyung , Kouis Panayiotis , Lam Yin Ting , Latzin Philipp , Legendre Marie , Lorent Natalie , Lucas Jane S. , Maitre Bernard , McEvoy Alison , Mitri-Frangieh Rana , Montani David , Müller Loretta , Muñoz Noelia , Omran Heymut , Ozcelik Ugur , Ozsezen Beste , Packham Samantha , Papon Jean-François , Pauly Clara , Pioch Charlotte , Poirrier Anne-Lise M.L. , Raidt Johanna , Reula Ana , Rinkel Rico , Roehmel Jobst , Schramm Andre , Thouvenin Guillaume , Walker Woolf T , Wilkins Hannah , Yiallouros Panayiotis , Yumusakhuylu Ali Cemal , Ziegahn Niklas 
Published: May 2023

Background

Sinonasal symptoms are a common feature of primary ciliary dyskinesia (PCD); however, literature about their severity and frequency, particularly during the life course, is scarce. Using baseline data from the Ear, nose and throat (ENT) Prospective International Cohort of PCD patients, we describe sinonasal disease in PCD.

Methods

We included participants who had a routine sinonasal examination during which they completed a symptoms questionnaire. We compared frequency of reported symptoms and examination findings among children and adults, and identified characteristics potentially associated with higher risk of sinonasal disease using ordinal regression.

Results

12 centres contributed 384 participants; median age was 16 years (IQR 9–22), and 54% were male. Chronic nasal problems were the most common feature, reported by 341 (89%). More adults (33; 24%) than children (10; 4%) described hyposmia. Quality of life was moderately affected by rhinosinusitis among 136 participants with completed SNOT-22 questionnaires (median score 31; IQR 23–45). Examinations revealed nasal polyps among 51 of 345 participants (15%) and hypertrophic inferior nasal turbinates among 127 of 341 participants (37%). Facial pain was detected in 50 of 342 participants (15%). Nasal polyps, hypertrophic turbinates, deviated septum and facial pain were found more commonly in adults than children. The only characteristic associated with higher risk of sinonasal disease was age 10 years and older.

Conclusions

Based on our findings, regular sinonasal examinations are relevant for patients with PCD of all ages. There is a need for improved management of sinonasal disease supported by evidence-based guidelines.