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.