Abstract：
Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibro-inflammatory disease that may cause dysfunction in various organs. Worldwide multidisciplinary experts attending the Fourth International Symposium on IgG4-Related Disease in Japan in 2021 discussed treatments for IgG4-RD, especially glucocorticoid (GC) therapy. This review describes the efficacy, safety, and cost of treatments for IgG4-RD based on findings presented at the international symposium. A medium dose of GC was considered appropriate for the initial treatment of IgG4-RD. A randomized controlled trial and an open-label prospective study have shown that long-term maintenance GC therapy (prednisolone ≥ 5 mg/day) could prevent disease relapse. In addition, two open-label randomized controlled trials reported the effects of combinational use of GC and synthetic immunosuppressive agents, mycophenolate mofetil and leflunomide, on relapse prevention. Moreover, an open-label single-arm study showed an excellent rate of clinical response to rituximab. Many observational studies have shown the efficacy of an appropriate GC regimen in patients with IgG4-RD. Synthetic immunosuppressive agents and a molecular-targeted agent can be potent alternatives to GCs, but additional studies are required comparing their efficacy, risk of infection, and costs.
摘要：
免疫球蛋白G4相关疾病（IgG4-RD）是一种慢性纤维炎症性疾病，可能导致各种器官功能障碍。参加2021年日本第四届IgG4相关疾病国际研讨会的全球多学科专家讨论了IgG4-RD的治疗方法。尤其是糖皮质激素（GC）治疗。这篇综述描述了其疗效，安全,根据国际研讨会上发表的研究结果，IgG4-RD的治疗费用。认为中等剂量的GC适合于IgG4-RD的初始治疗。一项随机对照试验和一项开放标签的前瞻性研究表明，长期维持GC治疗（泼尼松龙≥5mg/天）可以预防疾病复发。此外,两项开放标签的随机对照试验报道了GC和合成免疫抑制剂联合使用的效果，霉酚酸酯和来氟米特，关于预防复发。此外，一项开放标签的单臂研究显示,利妥昔单抗的临床缓解率优异.许多观察性研究表明，适当的GC方案对IgG4-RD患者的疗效。合成免疫抑制剂和分子靶向剂可以是GC的有效替代品，但是需要更多的研究来比较它们的功效，感染的风险，和成本。
