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Background: Primary ciliary dyskinesia (PCD) is considered a rare cause of chronic rhinosinusitis with nasal polyposis (CRSwNP), which is reported in 6% of children with PCD. The forms of PCD associated with the variants of the GAS8 gene identified so far seem to be linked to recurrent respiratory infections (sinusitis, otitis, and bronchiectasis) without situs inversus. Case presentation: We report a case of an 11-year-old girl with recurrent otitis media, productive cough, and chronic rhinosinusitis with nasal polyposis with homozygosity for a novel nonsense mutation in the GAS8. Conclusion: Children with CRSwNP should be treated in a multidisciplinary manner (ENT, pulmonologist, allergist, pathologist, pediatrician, and geneticist) because nasal polyposis often hides etiologies that must be recognized.
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Background: Chronic rhinosinusitis with nasal polyposis (CRSwNP) is a frequently diagnosed chronic disease that has been widely recognized as a significant economic strain on society. Recently, concern about the rising prevalence of eosinophilia in CRSwNP has attracted research interest. A comprehensive understanding of the characteristics and disease burden of CRSwNP patients may contribute to improved management of these patients. Methods: We conducted a multi-center retrospective observational study based on real-world data. Patients were filtered into three groups: CRSwNP overall group, CRSwNP surgical group, and CRSwNP patients who had a relapse after surgeries. Furthermore, we used laboratory test results of blood eosinophil percentage (EOS%) as an indicator of eosinophilia. The comorbidities and medications of patients in the high and low EOS% groups were compared. Disease burden was measured from two aspects: direct costs and loss of working days. Results: A total of 1,724 CRSwNP patients were eligible, 527 of which were filtered into the surgical groups. Only 16 patients in this study were found to have a relapse. The mean ages of the CRSwNP non-surgical, CRSwNP surgical, and CRSwNP relapse groups were 46, 47, and 52.5 years old, respectively. Most patients sought treatment in the otolaryngology department. Among all three groups, the most prevalent comorbidities were allergic rhinitis and asthma. The most prescribed drug was intranasal/oral corticosteroids. The direct costs per person/year for the non-surgical group, surgical group, and relapse group were ¥188.60, ¥15,190.00, and ¥14,160.00, respectively. The loss of working days per person/year for the non-surgical group, surgical group, and relapse group was 10.41, 24.40, and 21.65 days, respectively. Conclusions: CRSwNP is a disease of middle age. It is frequently associated with asthma and allergic rhinitis. Eosinophilia was found to have a considerate influence on patients' treatment patterns. Based on available data, we noted that patients in the high EOS% group were more likely to have asthma and allergic rhinitis. Intranasal/oral corticosteroids were the most frequently used drug for patients with CRSwNP, while a smaller proportion of patients in the high EOS% group used intranasal/oral corticosteroids. The disease burden of CRSwNP is posing significant challenges to both patients and society.
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BACKGROUND: Chronic rhinosinusitis with nasal polyposis (CRSwNP) is an inflammatory condition of the upper airways. Optimal management is unclear. OBJECTIVE: We compared the effects of mAbs and aspirin desensitization (ASA-D) for treatment of CRSwNP. METHODS: We searched the Medline, Embase, Cochrane Central Register of Controlled Trials, International Clinical Trials Registry Platform, US Food and Drug Administration, and the European Medicines Agency databases from inception to August 4, 2021, for randomized controlled trials comparing the effects of mAbs and ASA-D for CRSwNP. We conducted network meta-analysis of sinusitis symptoms, heath-related quality of life, rescue oral corticosteroids and surgery, endoscopic and radiologic scores, and adverse events. We used the Grades of Recommendation Assessment, Development and Evaluation (GRADE) approach to assess certainty of evidence. PROSPERO CRD42020177334. RESULTS: Twenty-nine randomized controlled trials evaluating 8 treatments (n = 3461) were included in the network meta-analysis. Compared to placebo, moderate to high certainty evidence showed that health-related quality of life (SNOT-22) improved with dupilumab (mean difference [MD] -19.91 [95% confidence interval (CI) -22.50, -17.32]), omalizumab (MD -16.09 [95% CI -19.88, -12.30]), mepolizumab (MD -12.89 [95% CI -16.58, -9.19], ASA-D (MD -10.61 [95% CI -14.51, -6.71]), and benralizumab (MD -7.68 [95% CI -12.09, -3.27]). The risk of rescue nasal polyp surgery likely decreased with dupilumab (risk difference [RD] -16.35% [95% CI -18.13, -13.48]), omalizumab (RD -7.40% [95% CI -11.04, -2.43]), mepolizumab (RD -12.33% [95% CI -15.56, -7.22]), and ASA-D (RD -16.00% [95% CI -19.79, 0.21]; all moderate certainty). Comparisons among agents show with moderate to high certainty that dupilumab ranks among the most beneficial for 7 of 7 outcomes, omalizumab for 2 of 7, mepolizumab for 1 of 7, and ASA-D for 1 of 7. CONCLUSIONS: Multiple biologics and ASA-D credibly improve patient-important outcomes, with clinically important differences in effects among agents; dupilumab uniquely ranks among the most beneficial for all outcomes studied.
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Antineoplásicos Inmunológicos , Pólipos Nasales , Sinusitis , Anticuerpos Monoclonales/uso terapéutico , Antineoplásicos Inmunológicos/uso terapéutico , Aspirina/efectos adversos , Enfermedad Crónica , Humanos , Pólipos Nasales/tratamiento farmacológico , Metaanálisis en Red , Omalizumab/uso terapéutico , Calidad de Vida , Sinusitis/tratamiento farmacológicoRESUMEN
BACKGROUND: There are no head-to-head studies for patients with aspirin-exacerbated respiratory disease (AERD) comparing any of the 5 Food and Drug Administration-approved respiratory biologic therapies. OBJECTIVE: Explore outcomes in subjects with AERD using biologic therapies in a real-world clinic setting. METHODS: A retrospective pilot study was conducted for subjects with AERD who had been prescribed omalizumab (anti-IgE), mepolizumab (anti-IL-5), reslizumab (anti-IL-5), benralizumab (anti-IL-5 receptor alpha [anti-IL-5Rα]), or dupilumab (anti-IL-4 receptor alpha [anti-IL-4Rα]). Clinical outcomes pre- versus postinitiation of biologic therapy were explored including symptoms, 22-item sino-nasal outcome test scores, systemic corticosteroid and antibiotic prescriptions, and emergency room visits related to AERD. RESULTS: Of the 74 subjects, 58.1% (n = 43) had used 1 biologic, though many (41.9%, n = 31) trialed more than 1 biologic. Of the 50 subjects who had used anti-IL-4Rα therapy, 98% (49 of 50) still had this therapy prescribed at study completion compared with 48.6% (17 of 35) and 26.9% (7 of 26) of those who used anti-IgE and anti-IL-5 and anti-IL-5 receptor alpha (anti-IL-5/IL-5Rα) therapy, respectively. Among those on anti-IL-4Rα therapy, there was a significant reduction in median total 22-item sino-nasal outcome test scores (51 to 19, P = .0002), corticosteroid bursts (2 to 0, P < .0001), and median number of antibiotic courses for respiratory disease (1 to 0, P = .0469) prebiologic versus postbiologic initiation. No statistically significant difference in those outcomes was observed for individuals on anti-IgE or anti-IL-5/IL-5Rα therapy. CONCLUSIONS: Anti-IL-4Rα therapy led to significantly higher rates of clinical improvement in AERD when compared with anti-IL-5/IL-5Rα and anti-IgE biologic therapies. Prospective studies would help clarify best practices for the use of biologic therapies in AERD.