幼年发病的系统性红斑狼疮:问题的现状(文献综述)

M. I. Kaleda, I. Nikishina
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引用次数: 0

摘要

幼年型系统性红斑狼疮(jSLE)患者占所有系统性红斑狼疮患者的25%。青少年系统性红斑狼疮与成人系统性红斑狼疮的主要区别在于遗传因素在发病机制中的作用更大、活动度更高、更早出现并发症以及需要更积极的免疫抑制治疗,因此我们可以将儿童期发病视为系统性红斑狼疮的一种特殊表型。研究 jSLE 的意义在于其临床表现的多变性、病程的不可预测性、早期诊断的困难性、器官损害的快速发展以及预后的不利性。文章从实用的角度介绍了有关 jSLE 诊断、分类、临床表现特征和治疗方法的最重要的现代数据。
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Systemic lupus erythematosus with juvenile onset: current status of the problem (literature review)
Patients with juvenile-onset systemic lupus erythematosus (jSLE) account for up to 25% of all SLE patients. The main difference between jSLE and SLE in adults is the greater role of genetic factors in the pathogenesis, higher activity, earlier development of complications and the need for more aggressive immunosuppressive therapy, which allows us to consider the onset of the disease in childhood as a special phenotype of SLE. The relevance of the study of jSLE arises from the variability of clinical manifestations and the unpredictability of the course, the difficulty of early diagnosis, the rapid development of organ damage and the unfavorable life prognosis.The article presents the most important modern data on the diagnosis, classification, features of the clinical picture and treatment approaches of jSLE from a practical point of view.
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