Objectives To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence.

Methods An international Task Force formed the questions for the systematic literature reviews (January 2018–December 2022), followed by formulation and finalisation of the statements after a series of meetings. A predefined voting process was applied to each overarching principle and recommendation. Levels of evidence and strengths of recommendation were assigned, and participants finally provided their level of agreement with each item.

Results The Task Force agreed on 5 overarching principles and 13 recommendations, concerning the use of hydroxychloroquine (HCQ), glucocorticoids (GC), immunosuppressive drugs (ISDs) (including methotrexate, mycophenolate, azathioprine, cyclophosphamide (CYC)), calcineurin inhibitors (CNIs, cyclosporine, tacrolimus, voclosporin) and biologics (belimumab, anifrolumab, rituximab). Advice is also provided on treatment strategies and targets of therapy, assessment of response, combination and sequential therapies, and tapering of therapy. HCQ is recommended for all patients with lupus at a target dose 5mg/kg real body weight/ day, considering the individual’s risk for flares and retinal toxicity. GC are used as ’bridging therapy’ during periods of disease activity; for maintenance treatment, they should be minimised to equal or less than 5mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation of ISDs (methotrexate, azathioprine, mycophenolate) and/or biological agents (anifrolumab, belimumab) should be considered to control the disease and facilitate GC tapering/discontinuation. CYC and rituximab should be considered in organ-threatening and refractory disease, respectively. For active lupus nephritis, GC, mycophenolate or low-dose intravenous CYC are recommended as anchor drugs, and add-on therapy with belimumab or CNIs (voclosporin or tacrolimus) should be considered. Updated specific recommendations are also provided for cutaneous, neuropsychiatric and haematological disease, SLE-associated antiphospholipid syndrome, kidney protection, as well as preventative measures for infections, osteoporosis, cardiovascular disease.

Conclusion The updated recommendations provide consensus guidance on the management of SLE, combining evidence and expert opinion.

目的：根据新出现的证据更新EULAR关于系统性红斑狼疮（SLE）管理的建议。

方法：由国际工作组提出系统性文献综述的问题（2018年1月至2022年12月），然后在一系列会议后制定并定稿声明。对每项总体原则和建议都采用了预定的投票程序。指定证据等级和推荐强度，参与者最终提供与每个项目的一致性水平。

结果：工作组就羟氯喹(HCQ)、糖皮质激素(GC)、免疫抑制药物(ISD)（包括甲氨蝶呤、麦考酚酯、硫唑嘌呤、环磷酰胺(CYC)）、钙调磷酸酶抑制剂（CNI、环孢素、他克莫司、沃孢霉素）和生物制剂（贝利尤单抗、阿尼鲁单抗、利妥昔单抗）。还提供了关于治疗策略和治疗靶点、缓解评估、联合和序贯治疗以及逐渐减量治疗的建议。考虑到个体的复发和视网膜毒性风险，建议所有狼疮患者以5 mg/kg实际体重/天的目标剂量接受HCQ治疗。GC在疾病活动期间用作“桥接治疗”；对于维持治疗，应尽量减少至≤5mg/天（泼尼松当量），并在可能的情况下停用。应考虑及时开始ISD（甲氨蝶呤、硫唑嘌呤、麦考酚酯）和/或生物制剂（阿尼鲁单抗、贝利尤单抗）治疗，以控制疾病并促进GC 逐渐减量/停药。CYC和利妥昔单抗应分别考虑用于危及器官和难治性疾病。对于活动性狼疮肾炎，推荐使用GC、麦考酚酯或低剂量静脉注射CYC作为锚定药物，并应考虑使用贝利尤单抗或CNI（伏孢霉素或他克莫司）进行辅助治疗。还针对皮肤、神经精神和血液学疾病、SLE相关抗磷脂综合征、肾脏保护以及感染、骨质疏松症、心血管疾病的预防措施提供了更新的具体建议。

结论：更新的建议结合证据和专家意见，为SLE的管理提供了共识指导。