Yamamoto Motohisa
Department of Rheumatology, Sapporo Medical University School of Medicine.
Nihon Rinsho Meneki Gakkai Kaishi. 2016;39(6):485-490. doi: 10.2177/jsci.39.485.
IgG4-related disease is a chronic and fibroinflammatory disorder, which is characterized with elevated levels of serum IgG4, and prominent infiltration of IgG4-bearing plasma cells in the involved organs. It often affects with lacrimal glands, salivary glands, pancreas, kidneys, lungs, and retroperitoneal cavity. Now, the first line of the induction therapy for IgG4-related disease is glucocorticoid, but almost patients need the maintenance treatment and experience the relapse. It is recently reported that biologic agents, including rituximab and abatacept, are effective for the relapse of IgG4-related disease. It is clear that the tapering effect of glucocorticoid is better than conventional oral immunosuppressants. We can use it in safely if we choose the appropriate cases. The investigator-initiated trial of rituximab for IgG4-related disease is scheduled in Japan. This article reviews the new strategies for the treatment of IgG4-related disease with our data of SMART registry, and discuss the problems of each biologic agents for IgG4-related disease.
IgG4相关性疾病是一种慢性纤维炎症性疾病，其特征为血清IgG4水平升高，以及受累器官中富含IgG4的浆细胞显著浸润。它常累及泪腺、唾液腺、胰腺、肾脏、肺和腹膜后腔。目前，IgG4相关性疾病诱导治疗的一线药物是糖皮质激素，但几乎所有患者都需要维持治疗并经历复发。最近有报道称，包括利妥昔单抗和阿巴西普在内的生物制剂对IgG4相关性疾病的复发有效。显然，糖皮质激素的减量效果优于传统口服免疫抑制剂。如果选择合适的病例，我们可以安全地使用它。在日本，由研究者发起的利妥昔单抗治疗IgG4相关性疾病的试验已在计划之中。本文结合我们SMART注册研究的数据，综述了IgG4相关性疾病的新治疗策略，并讨论了每种生物制剂在IgG4相关性疾病治疗中存在的问题。
