Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Japan
Department of Otolaryngology-Head and Neck Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan.
RMD Open. 2023 Mar;9(1). doi: 10.1136/rmdopen-2023-003026.
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.
度普利尤单抗（DUP）是一种针对白细胞介素（IL）-4 受体α的单克隆抗体，可抑制 IL-4 和 IL-13 信号通路，已被批准用于 2 型炎症性疾病，如哮喘、伴鼻息肉的慢性鼻-鼻窦炎和特应性皮炎；然而，由于基于几项病例报告的结果存在争议，DUP 治疗 IgG4 相关疾病（IgG4-RD）的疗效仍存在争议。在此，我们回顾了我院及既往文献中 4 例 IgG4-RD 患者使用 DUP 的疗效。所有使用 DUP 的患者均符合 2019 年 ACR/EULAR IgG4-RD 合并严重哮喘和伴鼻息肉的慢性鼻-鼻窦炎分类标准。2 例患者未使用全身糖皮质激素（GCs）接受 DUP 治疗，6 个月时颌下腺肿胀体积缩小约 70%。2 例接受 GCs 治疗的患者在 6 个月内成功减少了每日 GCs 剂量（分别减少 10%和 50%）。在所有 4 例患者中，6 个月时血清 IgG4 浓度和 IgG4-RD 应答指数均降低。
DUP 在 6 个月内可减少 IgG4-RD 合并严重哮喘或嗜酸性鼻-鼻窦炎患者颌下腺肿胀体积、血清 IgG4 水平、应答指数和 GCs 日剂量。由于病例报告有限且结果存在争议，DUP 治疗 IgG4-RD 的疗效仍存在争议。在此，我们报告了 2 例 IgG4-RD 患者在未使用全身 GCs 的情况下接受 DUP 治疗，显示颌下腺肿胀体积缩小，2 例患者显示 DUP 有 GC 节约作用。DUP 可改善 IgG4-RD 患者的疾病活动度并可作为类固醇节约剂。
