Division of Systemic Autoimmune Diseases, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
Division of Systemic Autoimmune Diseases, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain; Divisions of Rheumatology (London and Windsor campuses) and General Internal Medicine (Windsor Campus), Western University, London, Ontario, Canada.
Autoimmun Rev. 2023 Mar;22(3):103273. doi: 10.1016/j.autrev.2023.103273. Epub 2023 Jan 20.
IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition affecting multiple organs lacking standardized management. In this article, we review the evidence available to provide European expert-based statements on the management of IgG4-RD which were integrated in a final algorithm.
A panel of nine European experts in IgG4-RD from different specialties was asked to elaborate a set of consensus statements through a Delphi exercise. Three rounds of survey were taken. Consensus was reached when ≥75% of the responders agreed with a statement.
Thirty-one statements on induction treatment, maintenance treatment, non-pharmacological treatment, and general considerations were assessed. Patients should be treated promptly in situations when there is an immediate organ threatened, or when organ damage is anticipated. Glucocorticoids (GC) are considered the first line of treatment and should be progressively tapered. Maintenance treatment is recommended for patients with high disease activity or with risk factors for relapse. Rituximab is effective for induction and maintenance of remission, but its use can be limited by economics. Low dose GC with or without GC-sparing agents can be used for maintenance therapy. Stenting or surgery should be ancillary to pharmacological treatment. Follow up should be based on physical examination, blood works, and imaging studies. Furthermore, it should be tailored on individual patient clinical history. 18-fluorodeoxyglucose positron emission tomography/computerized tomography may provide additional information over other imaging modalities.
These new statements and algorithm reached a high degree of agreement and may help guiding the clinical management of IgG4-RD.
IgG4 相关疾病（IgG4-RD）是一种罕见的多器官纤维炎症性疾病，缺乏标准化的管理。本文回顾了现有的证据，为 IgG4-RD 的管理提供了基于欧洲专家的循证声明，并将其整合到最终的算法中。
我们邀请了来自不同专业的九位欧洲 IgG4-RD 专家组成专家组，通过 Delphi 研究对一系列共识声明进行了阐述。进行了三轮调查。当≥75%的应答者同意某项声明时，即可达成共识。
对诱导治疗、维持治疗、非药物治疗和一般注意事项等 31 项进行了评估。在存在危及器官的紧急情况或预期发生器官损害的情况下，应及时对患者进行治疗。糖皮质激素（GC）被认为是一线治疗药物，应逐渐减量。对于疾病活动度高或有复发风险的患者，建议进行维持治疗。利妥昔单抗对诱导和维持缓解有效，但由于经济原因，其应用可能受到限制。可以使用低剂量 GC 联合或不联合 GC 保留药物进行维持治疗。支架或手术应作为药物治疗的辅助手段。随访应基于体格检查、血液检查和影像学研究。此外，应根据个体患者的临床病史进行调整。18-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描（18-FDG PET/CT）可能比其他影像学方法提供更多的信息。
这些新的声明和算法达成了高度一致，可能有助于指导 IgG4-RD 的临床管理。
