Prevalence of chronic rhinosinusitis in bronchiectasis patients suspected of ciliary dyskinesia

Abstract

Background: Mucociliary clearance is a main defense mechanism of the airway and is impaired in ciliary dyskinesia. The objective of this study was to evaluate the prevalence of chronic rhinosinusitis (CRS) and its characteristics in bronchiectasis patients suspected of harboring ciliary dyskinesia. Methods:Bronchiectasis patients referred to a rhinology clinic for nasal brush biopsy (NBB) were included in this study. NBB was performed using a cure age technique whereby ciliated epithelial cells were obtained from the surface of the inferior nasal turbinate. Results of transmission electron microscopy findings, primary ciliary dyskinesia (PCD) gene (35 genes) analyses (Invitae), and sinus computed tomography (CT) scans were reviewed.Results: Twenty-three patients (age, 54 ± 2.9 years) were referred for NBB between 2015 and 2018. Thirteen patients (56.5%) met the criteria for diagnosis of CRS. Nineteen patients had ciliary ultrastructural defects. The most common finding was compound cilia (n = 11, 47.8%). Five patients (21.7%) had central microtubule defects (CMD) with higher forced expiratory volume in 1 second (FEV 1 ) at the time of referral than those without CMD (CMD + , 91 ± 3.7%; CMD − , 73.5 ± 5.7%; p = 0.023). Of 15 subjects with a PCD gene panel, 67% (9 of 15) carried at least 1 gene associated with PCD. Only 1 patient reached diagnosis of PCD. Approximately 50% of non-PCD carriers had a smoking history (p < 0.05). Lund-Mackay scores did not significantly differ between PCD and non-PCD carriers (p = 0.72). Conclusion:Nearly half of bronchiectasis patients referred for NBB had concurrent CRS. The presence of ciliary abnormalities was not amplified in bronchiectasis patients with CRS compared to those without CRS. Extrinsic factors may be related to ciliary structural abnormalities in non-PCD gene carriers. C 2019 ARS-AAOA, LLC.

Contemporary Classification of Chronic Rhinosinusitis Beyond Polyps vs No Polyps

“…Cystic fibrosis is a disorder of impaired mucociliary clearance. Primary ciliary dyskinesia is an example of loss of mechanical action of the cilia …”

The clinical impact of self‐reported symptoms of chronic rhinosinusitis in people with bronchiectasis

“…We included individuals with a primary diagnosis of bronchiectasis, including those with primary ciliary dyskinesia. 45 This, together with comorbid respiratory conditions, including asthma, COPD, allergens or gastro‐esophageal reflux disease, 46 , 47 smoking history, 48 and frequency of acute exacerbations of bronchiectasis, 9 are all factors which are associated with chronic rhinosinusitis and may have contributed to the symptoms reported on the SNOT‐22 questionnaire. In the absence of collating this information, the extent to which the underlying cause of bronchiectasis and comorbid conditions contributed to symptoms of chronic rhinosinusitis or quality of life scores is not clear.…”

Safety and Pharmacokinetics of a Ciprofloxacin and Azithromycin Stent for Chronic Rhinosinusitis

“…Chronic rhinosinusitis (CRS) is a common disease of the nose and paranasal sinuses characterized by persistent inflammation and bacterial infection of the mucosal surface. [1][2][3][4][5][6][7] Symptoms can include nasal congestion, facial pain or pressure, anosmia (decreased smell), and purulent drainage and occur more than 12 weeks. Several topical medications directly administered via nasal sprays, nasal irrigations, and nasal drops relieve the clinical symptoms of CRS.…”