本文由小咖机器人翻译整理

期刊来源：JAMA

原文链接：https://doi.org/10.1001/jama.2024.2315

摘要内容如下：

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。全世界大约有340万人被诊断为SLE。

观察

大约90%的SLE患者是女性。虽然没有统一接受的SLE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%。这些分类标准既包括临床因素，如发热、血细胞减少、皮疹、关节炎和蛋白尿，这可能是狼疮性肾炎的指征；以及免疫学指标，如SLE特异性自身抗体和低补体水平。大约40%的SLE患者发展为狼疮性肾炎，估计10%的狼疮性肾炎患者在10年后发展为终末期肾病。治疗的主要目标是达到疾病缓解或静止，定义为最小的症状、低水平的自身免疫性炎症标记物和最小的全身糖皮质激素需求，同时患者接受维持剂量的免疫调节或免疫抑制药物治疗。治疗目标包括减少由于疾病或治疗毒性引起的疾病恶化、住院和器官损伤。羟氯喹是治疗SLE的标准药物，可显著降低死亡率。除羟氯喹外，治疗是个体化的，使用免疫抑制剂，如硫唑嘌呤、霉酚酸酯和环磷酰胺，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治疗系统性红斑狼疮的药物：贝利木单抗（2011年用于治疗活动性系统性红斑狼疮，2020年用于治疗狼疮性肾炎）、Voclosporin（用于治疗狼疮性肾炎）和Anifrolumab（用于治疗活动性系统性红斑狼疮）。

结论和相关性

系统性红斑狼疮与免疫介导的多器官损害和死亡率增加有关。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。当需要时，额外的免疫抑制和生物治疗包括硫唑嘌呤、霉酚酸酯、环磷酰胺、贝利木单抗、Voclosporin和Anifrolumab。

英文原文如下：

Abstracts

Importance  Systemic 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.

Observations  Approximately 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 Relevance  Systemic 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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