Jeon Hyun-Seob, Jang Jae-Hyuk, Lee Youngsoo, Park Hae-Sim
Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Gyeonggi-do, 16499, South Korea.
Allergy Asthma Clin Immunol. 2023 Aug 5;19(1):67. doi: 10.1186/s13223-023-00825-z.
Aspirin-exacerbated respiratory disease (AERD) and IgG4-related disease (IgG4RD) share a common pathway of Th2-mediated immune mechanism; there have been several cases of IgG4RD developed in patients with asthma, especially in those comorbid with chronic rhinosinusitis (CRS). IgG4RD has often been treated with systemic corticosteroids, rituximab, or immune-suppressive agents, but frequently failed with relapse.
Here, we present a case of a 64-year-old male patient with severe AERD with CRS complicated with IgG4RD, who has been successfully treated and maintained with anti-IL-4 receptor antibody, dupilumab after achieving unsatisfactory responses with previous treatments including steroids, rituximab, omalizumab, and reslizumab. The patient's symptoms (periorbital swelling and asthmatic/nasal symptoms) were remarkably improved; serum levels of IgG4/IgE as well as plasmablast/eosinophil counts progressively decreased without any recurrence sign for over 2 years of dupilumab treatment.
These findings demonstrate that blocking the IL-4/IL-13 pathway with dupilumab can be an effective treatment with long-term safety in patients with severe AERD with CRS complicated by IgG4RD.
阿司匹林加重性呼吸道疾病（AERD）和IgG4相关性疾病（IgG4RD）具有Th2介导的免疫机制这一共同途径；哮喘患者，尤其是合并慢性鼻-鼻窦炎（CRS）的患者中，已有数例发生IgG4RD。IgG4RD通常采用全身用糖皮质激素、利妥昔单抗或免疫抑制剂治疗，但常因复发而失败。
在此，我们报告一例64岁男性患者，患有重度AERD合并CRS及IgG4RD，在包括糖皮质激素、利妥昔单抗、奥马珠单抗和瑞利珠单抗在内的先前治疗效果不佳后，使用抗IL-4受体抗体度普利尤单抗成功治疗并维持病情。患者的症状（眶周肿胀及哮喘/鼻部症状）明显改善；在度普利尤单抗治疗超过2年的时间里，血清IgG4/IgE水平以及浆母细胞/嗜酸性粒细胞计数逐渐下降，且无任何复发迹象。
这些发现表明，对于重度AERD合并CRS及IgG4RD的患者，使用度普利尤单抗阻断IL-4/IL-13途径可能是一种有效的治疗方法，且具有长期安全性。
