苯那利珠单抗替代单药治疗复发性哮喘为主的嗜酸性肉芽肿病伴多血管炎

IF 1.1 Q4 IMMUNOLOGY
M. Onuigbo, B. Libman, M. Lazarovich
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引用次数: 0

摘要

对类固醇耐药性或类固醇依赖性嗜酸性肉芽肿伴多血管炎(EGPA)的生物制品越来越感兴趣。我们描述了苯那利珠单抗单药治疗哮喘占主导地位的EGPA的成功。16年前,一名38岁男性被诊断患有哮喘、关节痛、贝尔氏麻痹和肾病综合征等多系统疾病,嗜酸性粒细胞增多20.3%(绝对计数1160 K/µL),ESR 50 mm/小时,白蛋白2.3 g/dL,血清肌酐1.1 mg/dL,略高于基线,免疫学检查呈阴性。肾活检显示弥漫性足细胞足突消失,病变轻微,无血管炎,诊断为EGPA。他通过皮质类固醇和环磷酰胺静脉注射获得早期缓解,并继续服用泼尼松和霉酚酸酯。然而,他反复经历严重的哮喘恶化、FEV-1%下降和复发性嗜酸性粒细胞增多症。他成功地开始服用benralizumab,并停止服用麦考酚酯和泼尼松。嗜酸性粒细胞增多症得到纠正,FEV-1%正常化。我们支持在EGPA中进行更大规模的生物制剂试验的呼吁。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Benralizumab substitution monotherapy in symptomatic relapsing asthma-dominant eosinophilic granulomatosis with polyangiitis
There is increasing interest in biologicals in steroid-resistant or steroid-dependent eosinophilic granulomatosis with polyangiitis (EGPA). We describe successful benralizumab monotherapy in asthma-dominant EGPA. Sixteen years ago, a 38-year-old male was diagnosed with multisystemic illness with asthma, arthralgias, Bell’s palsy and nephrotic syndrome, with 20.3% eosinophilia (absolute count 1160 K/µL), ESR 50 mm/hour, albumin 2.3 g/dL, serum creatinine 1.1 mg/dL, slightly above baseline and negative immunology work. Kidney biopsy demonstrated diffuse podocyte foot process effacement with minimal change disease, without vasculitis and EGPA was diagnosed. He achieved early remission with corticosteroids and intravenous cyclophosphamide and was maintained on prednisone and mycophenolate mofetil. Nevertheless, he repeatedly experienced severe asthma exacerbations, falls in FEV-1% and recurrent relapsing eosinophilia. He was successfully started on benralizumab and was weaned off mycophenolate mofetil and prednisone. Eosinophilia was corrected and FEV-1% normalized. We support calls for larger trials of the biologicals in EGPA.
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来源期刊
CiteScore
1.70
自引率
0.00%
发文量
65
审稿时长
3 weeks
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