Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
Department of Internal Medicine, Saint Peter's University Hospital, Rutgers Robert Wood Johnson School of Medicine, New Brunswick, New Jersey.
Clin Gastroenterol Hepatol. 2018 Dec;16(12):1947-1953. doi: 10.1016/j.cgh.2018.02.049. Epub 2018 Mar 8.
BACKGROUND & AIMS: IgG4-related disease (IgG4-RD), a multi-organ fibroinflammatory syndrome, typically responds to steroids. However, some cases are steroid resistant, and pancreaticobiliary IgG4-RD commonly relapses after steroid withdrawal. Rituximab induces remission of IgG4-RD, but the need for and safety of maintenance rituximab treatment are unknown. We compared outcomes of patients with pancreaticobiliary IgG4-RD treated with or without maintenance rituximab therapy.
We performed a retrospective study of patients with pancreaticobiliary IgG4-RD treated with rituximab at the Mayo Clinic in Rochester, Minnesota, from January 2005 through December 2015. The cohort was divided into patients who received only rituximab induction therapy (group 1, n = 14) and patients who received rituximab induction followed by maintenance therapy (group 2, n = 29). We collected data on recurrence of IgG4-RD symptoms and findings, as well as information on evaluations, treatment, and adverse events.
Median follow-up times were similar between group 1 (34 mo) and group 2 (27 mo) (P = .99). Thirty-seven patients (86%) were in steroid-free remission 6 months after rituximab initiation. A higher proportion of patients in group 1 had disease relapse (3-year event rate, 45%) than in group 2 (3-year event rate, 11%) (P = .034). Younger age, higher IgG4 responder index score after induction therapy, and increased serum levels of alkaline phosphatase at baseline or after rituximab induction were associated with relapse. Infections developed in 6 of 43 patients, all in group 2 (P = .067 vs group 1); all but 1 occurred during maintenance therapy.
In a retrospective study of patients with pancreaticobiliary IgG4-RD, we found rituximab maintenance therapy prolongs remission. Relapses are uncommon among patients receiving maintenance therapy, but maintenance therapy may increase risk of infection. Patients with factors that predict relapse could be candidates for rituximab maintenance therapy.
IgG4 相关疾病（IgG4-RD）是一种多器官纤维炎症综合征，通常对类固醇有反应。然而，有些病例对抗固醇治疗有抵抗性，且胰胆管 IgG4-RD 通常在停用类固醇后复发。利妥昔单抗可诱导 IgG4-RD 缓解，但维持利妥昔单抗治疗的必要性和安全性尚不清楚。我们比较了接受或不接受维持性利妥昔单抗治疗的胰胆管 IgG4-RD 患者的结局。
我们对明尼苏达州罗切斯特市梅奥诊所 2005 年 1 月至 2015 年 12 月期间接受利妥昔单抗治疗的胰胆管 IgG4-RD 患者进行了回顾性研究。该队列分为仅接受利妥昔单抗诱导治疗的患者（第 1 组，n=14）和接受利妥昔单抗诱导后接受维持治疗的患者（第 2 组，n=29）。我们收集了 IgG4-RD 症状和发现复发、评估、治疗和不良事件的信息。
第 1 组（34 个月）和第 2 组（27 个月）的中位随访时间相似（P=.99）。37 例（86%）患者在利妥昔单抗起始后 6 个月时达到无类固醇缓解。第 1 组疾病复发的患者比例较高（3 年事件率为 45%），而第 2 组为 11%（P=.034）。年轻、诱导治疗后 IgG4 应答指数评分较高、基线或利妥昔单抗诱导后碱性磷酸酶水平升高与复发相关。43 例患者中有 6 例发生感染，均发生在第 2 组（P=.067 比第 1 组）；除 1 例外，所有感染均发生在维持治疗期间。
在一项回顾性研究中，我们发现胰胆管 IgG4-RD 患者接受利妥昔单抗维持治疗可延长缓解期。接受维持治疗的患者复发罕见，但维持治疗可能增加感染风险。具有预测复发因素的患者可能是利妥昔单抗维持治疗的候选者。
