{"title":"Dupilumab in the treatment of severe chronic rhinosinusitis with nasal polyps (CRSwNP) in cystic fibrosis patients.","authors":"Attilio Di Girolamo, Mattia Cristallo, Michelina Francesca Daddato, Federico Spataro, Giuseppina Leonetti, Pamela Vitullo, Eustachio Nettis","doi":"10.1016/j.jcf.2026.04.003","DOIUrl":null,"url":null,"abstract":"<p><p>Chronic rhinosinusitis with nasal polyps (CRSwNP) manifests as either a solitary upper airway involvement or as part of a more intricate clinical picture, such as Cystic Fibrosis (CF). The altered viscoelastic properties of mucus facilitate the colonisation of bacteria, thereby instigating a process that perpetuates a state of chronic inflammation. Dupilumab, a monoclonal antibody that blocks IL-4/IL-13-signalling, has been demonstrated to improve nasal obstruction and the sense of smell. This observational real-life study comprised 10 patients diagnosed with CRSwNP and CF. The following scores were considered to ascertain the severity of rhinosinusitis: Nasal Polyp Score (NPS), Sino-Nasal Outcome Test (SNOT-22 score), Nasal Congestion/Obstruction Symptom severity score (NCS), Visual Analogue Scale for rhinosinusitis (VAS), and Loss of Smell score (LOS). Moreover, spirometry parameters and the number of new infections/hospital admissions were considered for lung involvement. Blood parameters, including the number of eosinophils and the total serum IgE level, were assessed. After six months of therapy, the median value of the NPS score decreased from 4 (IQR 1.75) to 1.5 (IQR 4.5; P < 0.01) and the SNOT-22 score from 55 (IQR 8.75) to 13.5 (IQR 13.5; P < 0.0001). FEV1 improved from a median value of 2.28 L (IQR 1.26) to 2.54 L (IQR 1.02). Serum IgE and blood eosinophil count did not change significantly. Dupilumab has proven to be effective in reducing the size of nasal polyps and improving associated symptoms in patients with CF who have type 2inflammation-related comorbidities, without the occurrence of adverse events.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0000,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Cystic Fibrosis","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.jcf.2026.04.003","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
引用次数: 0
Abstract
Chronic rhinosinusitis with nasal polyps (CRSwNP) manifests as either a solitary upper airway involvement or as part of a more intricate clinical picture, such as Cystic Fibrosis (CF). The altered viscoelastic properties of mucus facilitate the colonisation of bacteria, thereby instigating a process that perpetuates a state of chronic inflammation. Dupilumab, a monoclonal antibody that blocks IL-4/IL-13-signalling, has been demonstrated to improve nasal obstruction and the sense of smell. This observational real-life study comprised 10 patients diagnosed with CRSwNP and CF. The following scores were considered to ascertain the severity of rhinosinusitis: Nasal Polyp Score (NPS), Sino-Nasal Outcome Test (SNOT-22 score), Nasal Congestion/Obstruction Symptom severity score (NCS), Visual Analogue Scale for rhinosinusitis (VAS), and Loss of Smell score (LOS). Moreover, spirometry parameters and the number of new infections/hospital admissions were considered for lung involvement. Blood parameters, including the number of eosinophils and the total serum IgE level, were assessed. After six months of therapy, the median value of the NPS score decreased from 4 (IQR 1.75) to 1.5 (IQR 4.5; P < 0.01) and the SNOT-22 score from 55 (IQR 8.75) to 13.5 (IQR 13.5; P < 0.0001). FEV1 improved from a median value of 2.28 L (IQR 1.26) to 2.54 L (IQR 1.02). Serum IgE and blood eosinophil count did not change significantly. Dupilumab has proven to be effective in reducing the size of nasal polyps and improving associated symptoms in patients with CF who have type 2inflammation-related comorbidities, without the occurrence of adverse events.
慢性鼻窦炎伴鼻息肉(CRSwNP)表现为孤立的上呼吸道受累或更复杂的临床表现的一部分,如囊性纤维化(CF)。黏液粘弹性的改变促进了细菌的定植,从而引发了一个持续慢性炎症状态的过程。Dupilumab是一种阻断IL-4/ il -13信号传导的单克隆抗体,已被证明可以改善鼻塞和嗅觉。本观察性现实研究包括10例诊断为CRSwNP和CF的患者。考虑以下评分来确定鼻窦炎的严重程度:鼻息肉评分(NPS),鼻-鼻结局测试(SNOT-22评分),鼻塞/阻塞症状严重程度评分(NCS),鼻窦炎视觉模拟量表(VAS)和嗅觉丧失评分(LOS)。此外,肺量测定参数和新感染/住院人数被视为肺部受累。评估血液参数,包括嗜酸性粒细胞数量和血清总IgE水平。治疗6个月后,NPS评分中位数从4分(IQR 1.75)降至1.5分(IQR 4.5, P < 0.01), SNOT-22评分从55分(IQR 8.75)降至13.5分(IQR 13.5, P < 0.0001)。FEV1从中位数2.28 L (IQR 1.26)提高到2.54 L (IQR 1.02)。血清IgE和血嗜酸性粒细胞计数无明显变化。Dupilumab已被证明可有效减少伴有2型炎症相关合并症的CF患者的鼻息肉大小并改善相关症状,且未发生不良事件。
期刊介绍:
The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis. The journal also publishes news and articles concerning the activities and policies of the ECFS as well as those of other societies related the ECFS.