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系统性红斑狼疮

Systemic lupus erythematosus.

作者信息

Shaikh Maliha F, Jordan Natasha, D'Cruz David P

机构信息

Department of Rheumatology, Addenbrooke's Hospital, Cambridge, UK.

Louis Coote Lupus Unit, Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK.

出版信息

Clin Med (Lond). 2017 Feb;17(1):78-83. doi: 10.7861/clinmedicine.17-1-78.

Abstract

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease that is highly heterogeneous in its presentation. This can pose significant challenges for physicians responsible for the diagnosis and treatment of such patients. SLE arises from a combination of genetic, epigenetic and environmental factors. Pathologically, the disease is primarily driven by loss of immune tolerance and abnormal B- and T-cell function. Major organ involvement may lead to significant morbidity and mortality. Classification criteria for SLE have been developed largely for research purposes; however, these are also widely used in clinical practice. Antinuclear antibodies are the hallmark serological feature, occurring in over 95% of patients with SLE at some point during their disease. The mainstay of treatment is antimalarial drugs such as hydroxychloroquine, combined with corticosteroids and conventional immunosuppressive drugs. An increasing understanding of pathogenesis has facilitated a move towards the development of targeted biologic therapies, with the introduction of rituximab and belimumab into clinical practice.

摘要

系统性红斑狼疮(SLE)是一种慢性多系统自身免疫性疾病,其临床表现具有高度异质性。这给负责此类患者诊断和治疗的医生带来了重大挑战。SLE 由遗传、表观遗传和环境因素共同作用引发。病理上,该疾病主要由免疫耐受丧失以及 B 细胞和 T 细胞功能异常驱动。主要器官受累可能导致严重的发病率和死亡率。SLE 的分类标准主要是为研究目的而制定的;然而,这些标准在临床实践中也被广泛使用。抗核抗体是标志性的血清学特征,超过 95%的 SLE 患者在病程中的某个阶段会出现。治疗的主要药物是抗疟药,如羟氯喹,联合皮质类固醇和传统免疫抑制药物。对发病机制的深入了解推动了靶向生物疗法的发展,利妥昔单抗和贝利尤单抗已被引入临床实践。

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