Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

Krajewska, Joanna; Zub, Krzysztof; Slowikowski, A; Zatoński, Tomasz

doi:10.1007/s00405-021-06875-6

Cited by 14 publications

(13 citation statements)

References 78 publications

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“…Another poorly discussed question regarding OSA in adult pwCF is the role of the upper airways. CF results in impaired mucociliary clearance in the upper respiratory tract as well as the lungs, which may lead to the development of chronic rhinosinusitis (CRS) [ 40 ]. CRS may narrow the upper airways and impair breathing through the nose especially during sleep.…”

Section: Discussionmentioning

confidence: 99%

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

et al. 2022

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Background Obstructive sleep apnea (OSA), nocturnal hypoxemia and excessive daytime sleepiness (EDS) are common comorbidities in people with cystic fibrosis (pwCF). Most of the data showing this originates from children and adolescents. The aim of this study was to collect data on sleep parameters, EDS and pulmonary function from a large cohort of adult pwCF. Methods Full overnight polysomnography (PSG) was performed. EDS was determined using the Epworth Sleepiness Scale (ESS). Demographic and clinical data (body mass index [BMI], pulmonary function, capillary blood gases) were collected. Results A total of 52 adult pwCF were included (mean age 30.7 ± 8.0 years, mean percent predicted forced expiratory volume in 1 s [ppFEV1] of 52.1 ± 14.8). Overall AHI was in the normal range (4.5 ± 4.0/h); 21/52 pwCF (40%) had an apnea-hypopnea index > 5/h. Nocturnal hypoxemia was found in 25% of participants and this was associated with ppFEV1 (p = 0.014), awake oxygen saturation (SpO2; p = 0.021) and awake partial pressure of oxygen (pO2; p = 0.003); there were no significant differences in age, lung function and BMI were found for pwCF with versus without OSA (all p > 0.05). Eight pwCF (15%) had an ESS score > 10 (indicating EDS). OSA was best predicted by awake pO2 (area under the curve [AUC] 0.66, p = 0.048), while nocturnal hypoxemia was best predicted by ppFEV1 (AUC 0.74, p = 0.009), awake pO2 (AUC 0.76, p = 0.006) and awake SpO2 (AUC 0.71; p = 0.025). Conclusion OSA, nocturnal hypoxemia and EDS were common in adult pwCF, but no strong predictors were identified. Therefore, we suggest regular PSG and ESS scoring in adult pwCF, regardless of disease severity.

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Section: Discussionmentioning

confidence: 99%

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

et al. 2022

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“…As regards clinical implication of our findings, this study suggests a possible CFTR modulator therapy efficacy in order to reduce rhinosinusal involvement in CF patients. Moreover, as reported by previous studies, CFTR modulator therapy could reduce sinonasal symptoms too, improving quality of life [ 15 16 17 ].…”

Section: Discussionmentioning

confidence: 68%

Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Tagliati

Pantano

Lanni

et al. 2022

Journal of the Belgian Society of Radiology

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Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have not been so well reported. A previous study reported that ivacaftor improves appearance of sinus disease on Computed Tomography (CT) in cystic fibrosis (CF) patients with G551D mutation. The aim of this study was to evaluate the effect of CFTR modulator therapy in sinus disease using CT scores in a wider CF patient population. Materials and Methods: Forty-eight adult patients with CF underwent at least one CT sinus examination before CFTR modulator therapy (ivacaftor, lumacaftor, ivacaftor/lumacaftor or elexcaftor/tezacaftor/ivacaftor) and one CT sinus examination one year after CFTR modulator therapy initiation. Two radiologists assessed the images in consensus. The Lund-Mackay score (LM score) and the Sheikh-Lind CT sinus disease severity scoring system (SL score) were used. The 22-item SinoNasal Outcome Test (SNOT-22) questionnaire was evaluated before CFTR modulator therapy and one year after CFTR modulator therapy initiation. Results: CT sinus examination after CFTR modulator therapy showed statistically significant lower mean LM, SL and SNOT-22 scores than CT sinus examination before CFTR modulator therapy (p < 0.001). Conclusion: Evolution of imaging findings on CT during follow-up closely correlate with improved SNOT-22 score one year after CFTR modulator therapy initiation, indicating that CT may be a useful adjunct during follow-up of CF patients under this treatment as an objective measure of sinonasal disease improvement.

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“…Future studies should exploit other treatments for CF‐CRS, especially in patients that are not eligible for modulators. Nasal irrigations and use of INCS are performed in 25% and 45% of the PwCF in our cohort, but to date, none of these agents have been recommended for CF‐CRS 11,31 . The difference in pathophysiology and high rates of bacterial colonization with the presence of MDR strains plays an important role in the treatment response 28 .…”

Section: Discussionmentioning

confidence: 98%

Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators

et al. 2023

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ObjectivesChronic rhinosinusitis (CRS) is prevalent in people with cystic fibrosis (PwCF) and is often refractory to treatments. Uncontrolled CRS might negatively impact the lower airways and the quality of life. The aim of this study is to evaluate the burden of cystic fibrosis (CF)‐related CRS in the era of CF transmembrane conductance regulator (CFTR) modulators.MethodsAdult PwCF were asked to fill in a questionnaire on sinonasal complaints, they underwent a nasal endoscopy, bacteriological sampling, and a CT scan. Afterwards, these outcome measures were compared between patients treated with and without modulators.ResultsIn the 122 included patients, CRS was present in 83%. CFTR modulators were prescribed in 48% of the patients, with a median of 10 months since the start of the treatment. Subjectively, the median SNOT‐22 score was 16/110. Objectively, a median Lund‐Kennedy score of 6/12 and modified Lund‐Mackay score of 10/24 were observed. No correlation could be found between SNOT‐22 score and other outcome measures including endoscopy and radiology. Altogether, 21% of the patients had controlled disease. When comparing patients treated with and without modulators, significantly lower CT scores (p = 0.0018) and less bacterial colonization (p = 0.0082) were observed in patients receiving modulators.ConclusionCF‐CRS is highly prevalent in our cohort and only the minority of PwCF has a well‐controlled disease. A multidisciplinary ENT‐pneumology clinic would be beneficial, as there is a high discrepancy between patient‐reported symptoms and the extent of the disease. CFTR modulators are promising, as lower CT scores and less bacterial colonization were observed in the modulator group.Level of EvidenceLevel 3 Laryngoscope, 2023

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Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

Cited by 14 publications

References 78 publications

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators

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