Cystic fibrosis-related chronic rhinosinusitis: the key role of a comprehensive evaluation in the era of highly effective modulator therapy.

IF 1.9 3区医学 Q2 OTORHINOLARYNGOLOGY

European Archives of Oto-Rhino-Laryngology Pub Date : 2024-12-01 Epub Date: 2024-08-07 DOI:10.1007/s00405-024-08888-3

Alberto Minzoni, Luca Mazzetti, Pietro Orlando, Giuseppe Licci, Giovanni Taccetti, Silvia Bresci, Giandomenico Maggiore

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Abstract

Background: Chronic rhinosinusitis (CRS) is prevalent in cystic fibrosis (CF), significantly affecting quality of life. The introduction of CFTR modulators, including elexacaftor-tezacaftor-ivacaftor (ETI), offers promise for improving sinonasal outcomes.

Methods: We conducted a retrospective cohort multicenter study analyzing electronic medical records of 45 adult CF patients with CRS, predominantly heterozygous for the ΔF508 mutation, treated with ETI between January 2018 and December 2023. Assessments included Sinonasal Outcome Test 22 (SNOT-22), Nasal Polyp Score (NPS), modified Lund-Kennedy Score (mLKS), Lund-Mackay Score (LMS), and olfactory function using smell loss visual analog scale (VAS) and Sniffin' Sticks identification test (SSIT).

Results: After 12 months of ETI therapy, significant improvements were observed in pulmonary function parameters (FEV1, FVC), CRS severity scores (SNOT-22, NPS, mLKS), radiological findings (LMS), and olfactory function. Subgroup analysis suggested enhanced efficacy in patients with prior endoscopic sinonasal surgery.

Conclusions: ETI therapy demonstrates comprehensive improvements in CRS and olfactory function in CF patients, highlighting the potential of CFTR modulators in managing sinonasal manifestations.

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囊性纤维化相关慢性鼻炎：高效调节剂疗法时代综合评估的关键作用。

背景：慢性鼻窦炎（CRS）是囊性纤维化（CF）患者的常见病，严重影响患者的生活质量。包括 elexacaftor-tezacaftor-ivacaftor (ETI) 在内的 CFTR 调节剂的引入为改善鼻窦症状带来了希望：我们开展了一项回顾性队列多中心研究，分析了 2018 年 1 月至 2023 年 12 月间接受 ETI 治疗的 45 名 CRS 成年 CF 患者的电子病历，这些患者主要为ΔF508 突变杂合子。评估内容包括鼻窦结果测试 22（SNOT-22）、鼻息肉评分（NPS）、改良伦德-肯尼迪评分（mLKS）、伦德-马凯评分（LMS），以及嗅觉功能（使用嗅觉损失视觉模拟量表（VAS）和嗅棒识别测试（SSIT））：经过 12 个月的 ETI 治疗后，肺功能参数（FEV1、FVC）、CRS 严重程度评分（SNOT-22、NPS、mLKS）、放射学检查结果（LMS）和嗅觉功能均有显著改善。亚组分析表明，曾接受过内窥镜鼻窦手术的患者疗效更佳：ETI疗法全面改善了CF患者的CRS和嗅觉功能，凸显了CFTR调节剂在控制鼻窦表现方面的潜力。

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来源期刊

European Archives of Oto-Rhino-Laryngology 医学-耳鼻喉科学

CiteScore

5.30

自引率

7.70%

发文量

537

审稿时长

2-4 weeks

期刊介绍： Official Journal of European Union of Medical Specialists – ORL Section and Board Official Journal of Confederation of European Oto-Rhino-Laryngology Head and Neck Surgery "European Archives of Oto-Rhino-Laryngology" publishes original clinical reports and clinically relevant experimental studies, as well as short communications presenting new results of special interest. With peer review by a respected international editorial board and prompt English-language publication, the journal provides rapid dissemination of information by authors from around the world. This particular feature makes it the journal of choice for readers who want to be informed about the continuing state of the art concerning basic sciences and the diagnosis and management of diseases of the head and neck on an international level. European Archives of Oto-Rhino-Laryngology was founded in 1864 as "Archiv für Ohrenheilkunde" by A. von Tröltsch, A. Politzer and H. Schwartze.