Abstract：
Dupilumab (DUP) is a monoclonal antibody that acts on the interleukin (IL)-4 receptor alpha, which inhibits IL-4 and IL-13 signalling and is approved for type 2 inflammatory diseases such as asthma, chronic rhinosinusitis with nasal polyposis and atopic dermatitis; however, the efficacy of DUP to IgG4-related disease (IgG4-RD) is under discussion due to the controversial outcomes based on the several case reports. Here, we reviewed the efficacy of DUP in four consecutive patients with IgG4-RD in our institute and the previous literature.All patients administered DUP fulfilled the 2019 ACR/EULAR classification criteria for IgG4-RD complicated with severe asthma and chronic rhinosinusitis with nasal polyposis. Two cases were administered DUP without systemic glucocorticoids (GCs), and in 6 months, the volume of swollen submandibular glands (SMGs) was reduced by approximately 70%. Two cases receiving GCs successfully reduced their daily dose of GCs (10 and 50% reduction, respectively) with dupilumab in 6 months. In all four cases, serum IgG4 concentration and IgG4-RD responder index decreased in 6 months.DUP reduced the volume of the swollen SMGs, serum IgG4 levels, responder index and the daily dose of GCs in patients with IgG4-RD with severe asthma or eosinophilic rhinosinusitis in 6 months.The efficacy of DUP to IgG4-RD is under discussion due to the limited case reports with controversial outcomes. Here, we demonstrated that two patients with IgG4-RD treated by DUP without systemic GCs, showed volume reduction of swollen SMGs and two cases showed GC-sparing effects by DUP. DUP can ameliorate the disease activity and be a steroid-sparing agent in patients with IgG4-RD.
摘要：
Dupilumab（DUP）是一种单克隆抗体，作用于白细胞介素（IL）-4受体α，抑制IL-4和IL-13信号，并被批准用于2型炎症性疾病，如哮喘，慢性鼻-鼻窦炎伴鼻息肉病和特应性皮炎;然而,DUP对IgG4相关疾病(IgG4-RD)的疗效正在讨论中,因为根据几例病例报告,结果存在争议.这里,我们回顾了DUP在我们研究所的4例IgG4-RD患者中的疗效,以及之前的文献.所有接受DUP治疗的患者均符合2019年ACR/EULAR分类标准IgG4-RD合并重度哮喘和慢性鼻-鼻窦炎伴鼻息肉病。2例给予DUP而不给予全身性糖皮质激素(GC),6个月后,肿胀的下颌下腺（SMG）的体积减少了约70％。两例接受GC的患者成功减少了GC的每日剂量（减少10％和50％，分别）在6个月内使用dupilumab。在所有四种情况下，血清IgG4浓度和IgG4-RD反应指数在6个月内下降。DUP减少了肿胀的SMG的体积，血清IgG4水平，重度哮喘或嗜酸性粒细胞性鼻窦炎的IgG4-RD患者在6个月内的反应者指数和日GCs剂量。由于病例报告有限，结果有争议，DUP对IgG4-RD的疗效正在讨论中。这里,我们证明了两名IgG4-RD患者接受DUP治疗，没有全身性GCs，显示肿胀的SMG体积减少，2例显示DUP保留GC的作用。DUP可以改善IgG4-RD患者的疾病活动并成为类固醇保护剂。
