IgG4-related disease (IgG4-RD) is a rare fibroinflammatory, multisystemic condition with a relapsing-remitting progression.1 The level of serum IgG4 correlates with inflammatory activity and organ involvement.1 Glucocorticoids are first line for IgG4-RD, but there are numerous adverse effects with chronic use.2 Dupilumab is a monoclonal antibody that acts on the interleukin 4 (IL-4) receptor alpha, shared by the IL-4 and IL-13 receptors.1 IL-4 causes isotype switching from IgM to IgG4 and IL-13 is implicated in fibrosis.3 Thus, it was postulated by the authors to investigate dupilumab as a novel steroid-sparing treatment for IgG4-RD. A 67-year-old man with no known allergies and a history of sensory neural hearing loss, recurrent bronchitis, spinal stenosis, moderate positional obstructive sleep apnoea, asthma, atopic dermatitis (which caused swelling around his eyes) and allergic rhinoconjunctivitis underwent extensive investigations over the past 2 years due to suspected IgG4-RD. The patient’s initial complaint was pruritic erythematous lesions on the legs, arms, chest and palms. …

中文翻译：

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Dupilumab 作为 IgG4 相关疾病的新型类固醇保留疗法

IgG4 相关疾病 (IgG4-RD) 是一种罕见的纤维炎症性多系统疾病，具有复发-缓解进展。1 血清 IgG4 水平与炎症活动和器官受累相关。1 糖皮质激素是 IgG4-RD 的一线药物，但也有长期使用会产生许多不良反应。2 Dupilumab 是一种单克隆抗体，作用于由 IL-4 和 IL-13 受体共有的白细胞介素 4 (IL-4) 受体 α。1 IL-4 导致同种型从 IgM 转换为 IgG4并且 IL-13 与纤维化有关。3 因此，作者假设研究 dupilumab 作为 IgG4-RD 的一种新的类固醇节约治疗。一名 67 岁男性，无已知过敏史，有感觉神经性听力损失、复发性支气管炎、椎管狭窄、中度位置性阻塞性睡眠呼吸暂停、哮喘、由于疑似 IgG4-RD，特应性皮炎（导致眼睛周围肿胀）和过敏性鼻结膜炎在过去 2 年中进行了广泛调查。患者最初的主诉是腿部、手臂、胸部和手掌出现瘙痒性红斑。…