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

Abstract

BackgroundSinonasal 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.MethodsWe 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.Results12 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 (14%) and hypertrophic inferior nasal turbinates among 127 of 341 participants (34%). Facial pain was detected among 50 of 342 participants (13%). 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.ConclusionsBased 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.

Association between upper and lower respiratory disease among patients with primary ciliary dyskinesia: an international study

“…Interactions with allergic rhinitis might be possible; however, lack of detailed data on most participants precluded examining such a hypothesis. We believe the interactions to be small, since we previously found no associations between sinonasal disease and any particular season, especially not pollination seasons [ 3 ]. For some patients, coexisting posterior nasal drip explained the association of daily nasal symptoms with sputum production.…”

“…We received ethics approval from each participating centre and ethics committee for human research in accordance with local legislation. We obtained informed consent or assent from either participants or parents or caregivers of participants aged ≤14 years as described previously [ 3 , 15 ]. Our reporting conforms with the Strengthening the Reporting of Observational Studies in Epidemiology statement [ 16 ].…”

“…Participants were diagnosed at participating centres following ERS guidelines [ 22 ] as described in previous publications [ 3 , 15 ]. Ultrastructural defects were categorised based on the international consensus guideline for reporting transmission electron microscopy (TEM) results, which defined class 1 (hallmark, namely outer dynein arm defects, outer and inner dynein arm defects and microtubular disorganisation with inner dynein arm defects) and class 2 defects, such as central complex defects [ 23 ].…”

“…Nearly all patients with primary ciliary dyskinesia (PCD) report chronic nasal problems caused by poor mucociliary clearance, leading to mucus stagnation in upper and lower airways [ 1 – 3 ]. Clogged airways facilitate recurrent infections, chronic microbial colonisation and airway inflammation, leading further to chronic rhinosinusitis and bronchiectasis [ 4 , 5 ].…”

Lack of Correlation of Sinonasal and Otologic Reported Symptoms With Objective Measurements Among Patients With Primary Ciliary Dyskinesia: An International Study

“…Sinonasal and otologic symptoms are common among patients with primary ciliary dyskinesia (PCD) of all ages [ 1 - 3 ]. Since most PCD symptoms are nonspecific, patients with PCD may learn to live with their symptoms or perceive them as unbothersome; thus, they underreport symptoms during clinical visits.…”