Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Villarroel 170 08036, Spain.
Department of Pneumology and Allergy, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Eur J Intern Med. 2022 Jan;95:61-66. doi: 10.1016/j.ejim.2021.08.021. Epub 2021 Sep 14.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a relapsing disease with frequent glucocorticoid dependence. Mepolizumab has been demonstrated to reduce flares and spare glucocorticoids (GC). However, EGPA is a heterogeneous condition and the effects of mepolizumab on specific disease manifestations has not been completely delimitated.
To analyse the impact of mepolizumab on manifestations derived from small-vessel vasculitis, ENT (ear, nose and throat) symptoms, asthma, eosinophilic tissue infiltration and anti-neutrophil cytoplasmic antibody (ANCA) status in a single-centre cohort of EGPA patients.
Medical charts of EGPA patients treated with mepolizumab were retrospectively reviewed by the authors to describe demographics, clinical characteristics, steroid dose at the initiation of mepolizumab and during follow-up, flares, disease activity, damage accrual and laboratory results.
Among 56 patients with EGPA regularly controlled at our department, 11 patients were treated with mepolizumab because of corticodependence and unsatisfactory disease control. The mean time of treatment was 38 months (range: 3-66 months). Patients with persistent symptoms improved their asthma control, but 3 of them persisted with recurrent ENT symptoms in spite of treatment with mepolizumab. None of the patients developed vasculitic manifestations (cutaneous, neurological, gastrointestinal, renal) during treatment. All patients achieved a Birmingham Vasculitis Activity Score (BVAS) of 0 points at 12 months or earlier. In general, patients reduced the number of flares, which tended to be milder, and all related to asthma or ENT manifestations. The improvement in disease activity allowed notable glucocorticoid tapering.
嗜酸性肉芽肿性多血管炎（EGPA）是一种易复发疾病，常伴有糖皮质激素依赖。美泊利珠单抗已被证实可减少疾病发作并减少糖皮质激素（GC）的使用。然而，EGPA 是一种异质性疾病，美泊利珠单抗对特定疾病表现的影响尚未完全明确。
分析美泊利珠单抗对我院单中心队列 EGPA 患者小血管血管炎、耳鼻喉（ENT）症状、哮喘、嗜酸性组织浸润和抗中性粒细胞胞质抗体（ANCA）状态的影响。
作者回顾性分析了接受美泊利珠单抗治疗的 EGPA 患者的病历，以描述人口统计学、临床特征、美泊利珠单抗起始时和随访期间的皮质激素剂量、疾病发作、疾病活动度、损伤进展和实验室结果。
在我院定期接受治疗的 56 例 EGPA 患者中，有 11 例因皮质激素依赖和疾病控制不佳而接受美泊利珠单抗治疗。治疗平均时间为 38 个月（范围：3-66 个月）。持续性症状的患者改善了哮喘控制，但其中 3 例尽管接受了美泊利珠单抗治疗，但仍反复出现 ENT 症状。在治疗期间，没有患者出现血管炎表现（皮肤、神经、胃肠道、肾脏）。所有患者在 12 个月或更早时间达到 Birmingham Vasculitis Activity Score（BVAS）0 分。总体而言，患者减少了疾病发作次数，且发作程度较轻，均与哮喘或 ENT 表现相关。疾病活动度的改善允许显著减少糖皮质激素的用量。
