1Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, UK.
Lupus. 2013 Oct;22(12):1309-19. doi: 10.1177/0961203313502863.
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.
青少年发病系统性红斑狼疮（JSLE）占所有系统性红斑狼疮病例的 15-20%。虽然这种慢性复杂多系统自身免疫性疾病的特征高度可变，但儿童和青少年的疾病通常比成年人更为严重，随着时间的推移，疾病的损害也会累积得更多。JSLE 的女性患病率较低，与成人形式在主要器官表现模式上存在差异。几乎所有患有 JSLE 的儿童都使用皮质类固醇，其中大多数需要额外的免疫抑制药物。早期做出诊断并优化疾病控制对于确保儿童和青少年的正常发育至关重要。在这个年轻人群中，必须特别考虑疾病和治疗相关毒性的长期后果。目前缺乏儿科特异性对照试验，治疗策略通常以成人数据为指导。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全面、整体、专门的多学科方法来管理 JSLE。
