Background Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations. Methods Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations. Results Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40–60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons. Conclusions We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.

中文翻译：

---

2018 年 EULAR 大血管炎管理建议更新

背景 自 2009 年欧洲抗风湿病联盟 (EULAR) 发布大血管血管炎 (LVV) 管理建议以来，已经发布了几项相关的随机临床试验和队列分析，它们有可能改变临床护理，因此支持需要更新原始建议。方法 EULAR 工作组针对 EULAR 认可的建议使用 EULAR 标准化操作程序，对来自 13 个国家/地区的 20 位专家进行了系统的文献审查并征求意见。我们修改了现有的推荐并创建了新的推荐。结果 制定了三项总体原则和 10 项建议。我们建议应通过影像学或组织学确认疑似 LVV 的诊断。为了诱导活动性巨细胞动脉炎 (GCA) 或高安动脉炎 (TAK) 的缓解，应立即开始高剂量糖皮质激素治疗（40-60 毫克/天泼尼松等效）。我们建议对选定的 GCA 患者（难治性或复发性疾病，糖皮质激素相关不良事件或并发症的风险增加）使用托珠单抗进行辅助治疗。甲氨蝶呤可用作替代品。所有 TAK 患者应联合使用非生物糖皮质激素保留剂，而生物制剂可用于难治性或复发患者。我们不再推荐常规使用抗血小板或抗凝治疗来治疗 LVV，除非有其他指征。