嗜酸性肉芽肿性多血管炎伴鼻息肉的慢性鼻窦炎：通过美泊利单抗给药及辅助性内镜鼻窦手术（ESS）作用的真实世界多维分析

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中文摘要

背景： 嗜酸性肉芽肿性多血管炎（EGPA）是一种累及上、下呼吸道的罕见坏死性血管炎。尽管使用了生物制剂，鼻窦疾病仍常持续存在。关于抗IL-5治疗下耳鼻喉科疾病轨迹及内镜鼻窦手术（ESS）作用的证据仍然有限，且常缺乏标准化评估工具。本研究对生物治疗下的鼻窦结局进行了系统、多维度的评估，并分析了ESS在真实世界队列中的作用。
方法： 我们回顾性分析了52例接受美泊利单抗治疗并随访24个月的EGPA患者。鼻窦疾病通过鼻息肉评分（NPS）、Lund-Kennedy评分（LKS）、Lund-Mackay评分（LMK）和SNOT-22进行评估，而全身活动性通过伯明翰血管炎活动评分（BVAS）进行评估。患者按美泊利单抗方案（100 mg、300 mg和降阶梯）进行分层。通过多因素logistic回归探索辅助性ESS的预测因素。
结果： 超过90%的患者出现全身缓解，嗜酸性粒细胞显著抑制（中位数700 → 59.5个细胞/µL）且BVAS = 0。相反，NPS和LKS保持稳定[IQR]：（NPS 4 [3-5] → 4 [2-5]；LKS 6 [4-8] → 5 [2-7.75]。64%的患者息肉持续存在，92%的人群存在黏膜水肿/分泌物。持续100 mg治疗显示出最大的内镜改善。较高的基线鼻窦负荷、更广泛的手术史和美泊利单抗剂量变化与辅助性ESS（28.8%）相关，而BVAS和嗜酸性粒细胞与耳鼻喉科严重程度无相关性。
结论： 尽管全身缓解，鼻窦疾病仍可能持续存在，提示存在部分独立的炎症微环境。综合评估应结合全身标志物与专门的耳鼻喉科评估，而慢性结构性疾病的管理可能需要生物-手术整合策略。

英文摘要

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotizing vasculitis involving upper- and lower-airways. Despite biologics, sinonasal disease often persists. Evidence on ENT trajectories under anti-IL5 treatment and role of endoscopic sinus surgery (ESS) remains limited and is often described without standardized assessment tools. This study provides a systematic, multidimensional evaluation of sinonasal outcomes under biologic therapy and analyzes the role of ESS in a real-life cohort.
METHODS: We retrospectively analyzed 52 EGPA patients with a 24-month follow-up on mepolizumab. Sinonasal disease was assessed by Nasal Polyp Score (NPS), Lund-Kennedy Score (LKS), Lund-Mackay score (LMK), and SNOT-22, while systemic activity was assessed by Birmingham Vasculitis Activity Score (BVAS). Patients were stratified by mepolizumab regimen (100 mg, 300 mg, and step-down). Predictors of adjunctive ESS were explored through multivariate logistic regression.
RESULTS: Systemic remission occurred in >90% of patients, with marked eosinophil suppression (median 700 → 59.5 cells/µL) and BVAS = 0. Conversely, NPS and LKS remained stable [IQR]: (NPS 4 [3-5] → 4 [2-5]; LKS 6 [4-8] → 5 [2-7.75]. Polyps persisted in 64% and mucosal edema/discharge in 92% of population. Continuous 100 mg therapy showed the greatest endoscopic improvement. Higher baseline sinonasal burden, more extensive surgical history, and mepolizumab dose variation were associated with adjunctive ESS (28.8%), whereas BVAS and eosinophils showed no correlation with ENT severity.
CONCLUSION: Sinonasal disease may persist despite systemic remission, suggesting a partially independent inflammatory niche. Comprehensive assessment should combine systemic markers with dedicated ENT evaluation, while management may require integrated biologic-surgical strategies in chronic structural disease.