TY - JOUR
T1 - Sinonasal disease among patients with primary ciliary dyskinesia
T2 - An international study
AU - Lam, Yin Ting
AU - Papon, Jean-François
AU - Alexandru, Mihaela
AU - Anagiotos, Andreas
AU - Armengot, Miguel
AU - Boon, Mieke
AU - Burgess, Andrea
AU - Crowley, Suzanne
AU - Dheyauldeen, Sinan Ahmed D.
AU - Emiralioglu, Nagehan
AU - Eralp, Ela Erdem
AU - van Gogh, Christine
AU - Gokdemir, Yasemin
AU - Gunaydın, Onder
AU - Haarman, Eric G.
AU - Harris, Amanda
AU - Hayn, Isolde
AU - Ismail-Koch, Hasnaa
AU - Karadag, B. lent
AU - Kempeneers, C. line
AU - Kim, Sookyung
AU - Latzin, Philipp
AU - Lorent, Natalie
AU - Ozcelik, Ugur
AU - Pioch, Charlotte
AU - Poirrier, Anne-Lise M. L.
AU - Reula, Ana
AU - Roehmel, Jobst
AU - EPIC-PCD team
AU - Yiallouros, Panayiotis
AU - Goutaki, Myrofora
N1 - Funding Information: This study is funded by a Swiss National Science Foundation Ambizione fellowship (PZ00P3_185923). The authors participate in the BEAT-PCD (Better Experimental Approaches to Treat PCD) clinical research collaboration, supported by the European Respiratory Society, and most centres are members of the PCD core of ERN-LUNG (European Reference Network on Rare Respiratory Diseases). Funding information for this article has been deposited with the Crossref Funder Registry. Funding Information: Support statement: This study is funded by a Swiss National Science Foundation Ambizione fellowship (PZ00P3_185923). The authors participate in the BEAT-PCD (Better Experimental Approaches to Treat PCD) clinical research collaboration, supported by the European Respiratory Society, and most centres are members of the PCD core of ERN-LUNG (European Reference Network on Rare Respiratory Diseases). Funding information for this article has been deposited with the Crossref Funder Registry. Publisher Copyright: © The authors 2023.
PY - 2023/5/1
Y1 - 2023/5/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85160665345&partnerID=8YFLogxK
U2 - https://doi.org/10.1183/23120541.00701-2022
DO - https://doi.org/10.1183/23120541.00701-2022
M3 - Article
C2 - 37228283
SN - 2044-6055
VL - 9
JO - ERJ open research
JF - ERJ open research
IS - 3
M1 - 00701-2022
ER -