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