Children and adolescents with SLE: not just little adults
Children and adolescents with SLE: not just little adults
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DOI：
10.1177/0961203313502863
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发表时间：
2013-10-01
期刊：
影响因子：
2.6
通讯作者：
Beresford, M. W.
中科院分区：
文献类型：
--
作者：
Morgan, T. A.;Watson, L.;Beresford, M. W.
幼年型系统性红斑狼疮（JSLE）占所有SLE病例的15-20%。虽然这种慢性复杂的多系统自身免疫性疾病的特征是高度可变的，但儿童和青少年通常比成人更严重，并且随着时间的推移会产生更大的疾病损害。JSLE具有不太明显的女性优势，并且在主要器官表现的模式上与成人形式不同。皮质类固醇用于几乎所有儿童与JSLE沿着与大多数需要额外的免疫抑制药物。早期诊断和优化疾病控制对于确保儿童和青少年的正常发育不受阻碍至关重要。在这一年轻人群中，必须特别考虑疾病的长期后遗症和治疗相关毒性。目前缺乏针对儿童的对照试验，治疗策略通常以成人数据为指导。这种疾病及其治疗对儿童或青少年及其家庭的巨大心理和社会影响需要一种全面，整体，专业的多学科方法来管理JSLE。
Juvenile-onset systemic lupus erythematosus (JSLE) represents 15-20% of all SLE cases. Whilst features of this chronic complex multisystem autoimmune disorder are highly variable, children and adolescents generally present with a more severe illness than adults and accrue greater disease damage over time. JSLE has a less striking female preponderance and differs from the adult form in pattern of major organ manifestations. Corticosteroids are used in almost all children with JSLE along with the majority requiring additional immunosuppressive medications. Making the diagnosis early and optimizing disease control are essential to ensure that normal childhood and adolescent development is not impeded. In this young population, special consideration must be given to the long-term sequelae of the disease and treatment-related toxicity. There is a current lack of paediatric-specific controlled trials and treatment strategies are generally guided by adult data. The enormous psychological and social impact of the disease and its treatments upon the child or young person and their family necessitates a comprehensive, holistic, specialized multidisciplinary approach to managing JSLE.