ImportanceSystemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by inflammation and immune-mediated injury to multiple organ systems, including the mucocutaneous, musculoskeletal, hematologic, and kidney systems. Approximately 3.4 million people worldwide have received a diagnosis of SLE.ObservationsApproximately 90% of people with SLE are female. Although there are no uniformly accepted diagnostic criteria for SLE, the 2019 European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism)/American College of Rheumatology classification criteria developed for scientific study are an estimated 96.1% sensitive and 93.4% specific for SLE. These classification criteria include both clinical factors, such as fever, cytopenia, rash, arthritis, and proteinuria, which may be indicative of lupus nephritis; and immunologic measures, such as SLE-specific autoantibodies and low complement levels. Approximately 40% of people with SLE develop lupus nephritis, and an estimated 10% of people with lupus nephritis develop end-stage kidney disease after 10 years. The primary goal of treatment is to achieve disease remission or quiescence, defined by minimal symptoms, low levels of autoimmune inflammatory markers, and minimal systemic glucocorticoid requirement while the patient is treated with maintenance doses of immunomodulatory or immunosuppressive medications. Treatment goals include reducing disease exacerbations, hospitalizations, and organ damage due to the disease or treatment toxicity. Hydroxychloroquine is standard of care for SLE and has been associated with a significant reduction in mortality. Treatments in addition to hydroxychloroquine are individualized, with immunosuppressive agents, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, typically used for treating moderate to severe disease. Three SLE medications were recently approved by the Food and Drug Administration: belimumab (for active SLE in 2011 and for lupus nephritis in 2020), voclosporin (for lupus nephritis), and anifrolumab (for active SLE).Conclusions and RelevanceSystemic lupus erythematosus is associated with immune-mediated damage to multiple organs and increased mortality. Hydroxychloroquine is first-line therapy and reduces disease activity, morbidity, and mortality. When needed, additional immunosuppressive and biologic therapies include azathioprine, mycophenolate mofetil, cyclophosphamide, belimumab, voclosporin, and anifrolumab.

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系统性红斑狼疮

重要性系统性红斑狼疮 (SLE) 是一种慢性自身免疫性疾病，其特征是炎症和免疫介导的多器官系统损伤，包括皮肤粘膜、肌肉骨骼、血液和肾脏系统。全球大约有 340 万人被诊断为 SLE。观察结果大约 90% 的 SLE 患者是女性。尽管 SLE 没有统一接受的诊断标准，但为科学研究制定的 2019 年欧洲风湿病协会联盟（前身为欧洲抗风湿病联盟）/美国风湿病学会分类标准估计对 SLE 的敏感性为 96.1%，特异性为 93.4% 。这些分类标准包括临床因素，如发烧、血细胞减少、皮疹、关节炎和蛋白尿，这些可能表明狼疮性肾炎；以及免疫学措施，例如 SLE 特异性自身抗体和低补体水平。大约 40% 的 SLE 患者会发展为狼疮性肾炎，估计 10% 的狼疮性肾炎患者会在 10 年后发展为终末期肾病。治疗的主要目标是实现疾病缓解或静止，即在患者接受维持剂量的免疫调节或免疫抑制药物治疗时，症状最轻、自身免疫炎症标志物水平较低以及全身糖皮质激素需求量最低。治疗目标包括减少疾病恶化、住院治疗以及因疾病或治疗毒性引起的器官损伤。羟氯喹是 SLE 的标准治疗方法，可显着降低死亡率。除了羟氯喹之外，治疗也是个体化的，可以使用免疫抑制剂，例如硫唑嘌呤、吗替麦考酚酯和环磷酰胺，通常用于治疗中度至重度疾病。美国食品和药物管理局最近批准了三种 SLE 药物：belimumab（2011 年用于治疗活动性 SLE，2020 年用于狼疮性肾炎）、voclosporin（用于狼疮性肾炎）和 anifrolumab（用于活动性 SLE）。结论和相关性系统性红斑狼疮与系统性红斑狼疮相关免疫介导的多器官损伤和死亡率增加。羟氯喹是一线治疗药物，可降低疾病活动性、发病率和死亡率。需要时，额外的免疫抑制和生物疗法包括硫唑嘌呤、吗替麦考酚酯、环磷酰胺、贝利木单抗、沃罗孢素和阿尼鲁单抗。