Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
Int J Rheum Dis. 2019 Aug;22(8):1479-1488. doi: 10.1111/1756-185X.13633. Epub 2019 Jun 27.
The purpose of this study is to evaluate the therapeutic efficacy and safety of iguratimod plus corticosteroid as bridge therapy in the treatment of mild immunoglobulin G4-related disease (IgG4-RD).
Newly diagnosed IgG4-RD patients, without internal organ involvement were enrolled. Patients were given one dose of diprospan, intramuscular injection, and iguratimod, 25 mg, twice daily, for 24 weeks and were followed up at 0, 12 and 24 weeks. Follow-up indexes included IgG4-RD responder index (IgG4-RD RI), serology and imaging, plasma cytokines and adverse drug effect. Flow cytometry was performed for T, B cell subsets and plasma was collected for liquid chromatography mass spectrometry (LC-MS)-based metabolomic profiling and data processing.
Thirty patients were enrolled. At week 24, 9 (30.0%) patients achieved complete response, 17 (56.7%) patients with partial response, and 4 (13.3%) patients had no response to treatment. IgG4-RD RI, serum IgG and IgG4 levels decreased significantly at weeks 12 and 24 after treatment, as well as CD3+ CD8+ T cells, plasmablast/plasma cells and memory B cells. The LC-MS based plasma metabolomic profiles revealed significant changes between untreated patients and healthy donors, which became much similar to normal states after treatment.
Iguratimod plus corticosteroid as bridge therapy is effective for the treatment of mild IgG4-RD, it can improve the clinical symptoms, reduce serum IgG and IgG4 levels, especially plasmablasts/plasma cells and memory B cells. In addition, the metabolite profiling became similar to normal controls after treatment.
本研究旨在评估来氟米特联合皮质类固醇作为桥接疗法治疗轻度免疫球蛋白 G4 相关疾病（IgG4-RD）的疗效和安全性。
纳入新诊断的 IgG4-RD 患者，无内脏器官受累。患者给予地塞米松磷酸钠注射液 1 次，肌内注射，同时来氟米特，25mg，每日 2 次，治疗 24 周，并在 0、12 和 24 周时进行随访。随访指标包括 IgG4-RD 应答指数（IgG4-RD RI）、血清学和影像学、血浆细胞因子和药物不良反应。采用流式细胞术检测 T、B 细胞亚群，采集血浆进行基于液相色谱-质谱（LC-MS）的代谢组学分析和数据处理。
共纳入 30 例患者。治疗 24 周时，9 例（30.0%）患者完全缓解，17 例（56.7%）患者部分缓解，4 例（13.3%）患者无反应。治疗后 12 周和 24 周时，IgG4-RD RI、血清 IgG 和 IgG4 水平显著降低，CD3+CD8+T 细胞、浆母细胞/浆细胞和记忆 B 细胞也显著降低。基于 LC-MS 的血浆代谢组学图谱显示，未经治疗的患者与健康供者之间存在显著差异，治疗后与正常状态更为相似。
来氟米特联合皮质类固醇作为桥接疗法治疗轻度 IgG4-RD 有效，可改善临床症状，降低血清 IgG 和 IgG4 水平，特别是浆母细胞/浆细胞和记忆 B 细胞。此外，治疗后代谢组学图谱变得与正常对照相似。
