Immuno-Rheumatology Center, St. Luke's International Hospital.
Center for Clinical Epidemiology, St. Luke's International University, Tokyo.
Rheumatology (Oxford). 2021 Dec 1;60(12):5697-5704. doi: 10.1093/rheumatology/keab235.
Patients with IgG4-related disease (IgG4RD) usually require steroid-sparing agents due to relapse with tapering glucocorticoids (GC). We aimed to determine the efficacy and safety of mizoribine (MZR) among IgG4RD patients.
We retrospectively reviewed records of IgG4RD patients at Immuno-Rheumatology Center in St. Luke's International Hospital, Tokyo, Japan. Patients treated with MZR were classified into the MZR group, and those treated with GC alone or with other immunosuppressants were included in the control group. Disease exacerbation, GC dose, IgG-IgG4 titre and adverse events were evaluated using univariate analyses, including the Kaplan-Meier method. The Cox proportional hazard model was used to evaluate risk factors for disease exacerbation.
A total of 14 and 29 cases were included in the MZR and control group. Multiple organ involvement (three or more organs) was significantly more frequent in the MZR group [10 (71.4%) vs 9 (31.0%), P= 0.021]. Kaplan-Meier analysis revealed a significant reduction inexacerbation in patients with multiple organ involvement (P< 0.001) but not in total (P= 0.42). The adjusted hazard ratios of MZR use and multiple organ involvement for exacerbation were 0.34 (95%CI 0.12-1.01; P = 0.052) and 3.51 (95%CI 1.29-9.51; P= 0.014). The cumulative GC dose (mg per year, interquartile range) tended to be lower in the MZR group [1448 (1003-1642) vs 2179 (1264-3425); P= 0.09].
MZR decreased disease exacerbation among IgG4RD patients with multi-organ involvement and showed a steroid-sparing effect. MZR could be a treatment option for IgG4RD.
由于糖皮质激素（GC）减量会导致 IgG4 相关疾病（IgG4RD）复发，因此此类患者通常需要使用激素抑制剂。本研究旨在评估吗替麦考酚酯（MZR）在 IgG4RD 患者中的疗效和安全性。
我们回顾性分析了日本东京圣卢克国际医院免疫风湿中心 IgG4RD 患者的病历。接受 MZR 治疗的患者被归入 MZR 组，接受 GC 单药或其他免疫抑制剂治疗的患者归入对照组。采用单因素分析包括 Kaplan-Meier 法评估疾病加重、GC 剂量、IgG-IgG4 滴度和不良反应。采用 Cox 比例风险模型评估疾病加重的危险因素。
MZR 组和对照组分别纳入 14 例和 29 例患者。MZR 组多器官受累（≥3 个器官）的比例显著高于对照组[10（71.4%）vs 9（31.0%），P=0.021]。Kaplan-Meier 分析显示，多器官受累患者的病情恶化显著减少（P<0.001），但总体无显著减少（P=0.42）。MZR 使用和多器官受累与疾病加重的调整后危险比分别为 0.34（95%CI 0.12-1.01；P=0.052）和 3.51（95%CI 1.29-9.51；P=0.014）。MZR 组的累积 GC 剂量（mg/年，四分位距）[1448（1003-1642）vs 2179（1264-3425）]有降低趋势，但差异无统计学意义（P=0.09）。
MZR 可减少多器官受累的 IgG4RD 患者的疾病加重，并具有激素节省作用。MZR 可能是 IgG4RD 的一种治疗选择。
