Aggarwal Amita, Srivastava Puja
Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Int J Rheum Dis. 2015 Feb;18(2):182-91. doi: 10.1111/1756-185X.12419. Epub 2014 Jun 26.
About 20% of systemic lupus erythematosus (SLE) starts in childhood and children have less gender bias in favor of females as compared to adults. Systemic manifestations, nephritis, neuro-psychiatric disease and cytopenias are more common in children at presentation than adults. Since most children develop lupus in their early adolescence, dealing with the diagnosis of an unpredictable lifelong disease during this phase of life is challenging. Physicians must recognise specific medical and social needs of this age group, for optimal long-term outcome. Steroids and immunosuppressive drugs are the cornerstone for treatment in children as with adults with lupus. The outcome has improved considerably with these drugs and 10-year survival is nearly 90%. Due to longer life spans more damage accrues in children as compared to adults. Most of the drugs are associated with significant toxicity and the goal of having a drug which reduces disease activity and damage without hampering normal growth, development and fertility is still an elusive one. The current review focuses on clinical and immunological aspects of childhood SLE and how it differs from adulthood SLE.
约20%的系统性红斑狼疮（SLE）始于儿童期，与成人相比，儿童SLE的发病较少偏向女性。与成人相比，儿童SLE起病时出现全身表现、肾炎、神经精神疾病和血细胞减少更为常见。由于大多数儿童在青春期早期患上狼疮，在这一人生阶段应对这种不可预测的终身疾病的诊断具有挑战性。为了获得最佳的长期预后，医生必须认识到这个年龄组的特殊医疗和社会需求。与成人狼疮患者一样，类固醇和免疫抑制药物是儿童SLE治疗的基石。使用这些药物后，预后有了显著改善，10年生存率接近90%。由于寿命延长，与成人相比，儿童累积的损害更多。大多数药物都有明显的毒性，研发一种既能降低疾病活动度和损害，又不影响正常生长、发育和生育能力的药物，这一目标仍然难以实现。本综述重点关注儿童SLE的临床和免疫学方面，以及它与成人SLE的不同之处。
