Section of Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Int J Rheum Dis. 2013 Feb;16(1):56-60. doi: 10.1111/1756-185x.12020. Epub 2012 Dec 7.
To report the long-term outcome of Saudi children with systemic lupus erythematosus (SLE).
Charts of all children with SLE treated between 1990 and 2010 at King Faisal Specialist Hospital and Research Center Riyadh, were reviewed. The long-term outcome measured by pediatric adaptation of the Systemic Lupus International Collaborating Clinics American College of Rheumatology Damage Index (pSDI) and death related to SLE were determined. The data included: gender, age at disease onset, clinical features and treatment at last follow-up visit.
One hundred and fifty-two patients (129 girls and 23 boys) were included. The mean age at onset of SLE was 8.8 ± 2.6 years, while the mean age at diagnosis was 9.5 ± 2.6 years and the mean disease duration was 7.5 ± 4.6 years. All patients were treated with corticosteroid and immunosuppressive drugs. Eighty (52.6%) patients had damage with a mean SDI score of 1.3 ± 1.7. Damage accrual was mostly in the growth (26.8%), renal (17.1%) and neuropsychiatric (15.8%) domains. Due to progressive renal disease, 14 patients required dialysis; five of them underwent renal transplant. There were nine deaths related to SLE, eight of them due to infection. Based on logistic regression, patient disease damage was significantly associated with young age at disease onset and long disease duration. Similarly, death related to SLE was influenced by early-onset disease. In contrast, gender, disease duration and therapy did not affect the suggested outcome measures.
Our results are comparable to reports from other tertiary centers. Early-onset disease probably influences the long-term outcome of SLE in children. Infection remains an important cause of death in children with SLE.
报告沙特系统性红斑狼疮（SLE）儿童的长期结局。
回顾了 1990 年至 2010 年在利雅得法赫德国王专科医院和研究中心接受治疗的所有 SLE 患儿的病历。通过儿科系统性红斑狼疮国际合作临床中心美国风湿病学会损伤指数（pSDI）评估长期结局，并确定与 SLE 相关的死亡。数据包括：性别、发病年龄、临床特征和最后一次随访时的治疗。
共纳入 152 例患儿（129 例女孩和 23 例男孩）。SLE 发病的平均年龄为 8.8±2.6 岁，确诊时的平均年龄为 9.5±2.6 岁，疾病持续时间的平均年龄为 7.5±4.6 岁。所有患者均接受糖皮质激素和免疫抑制剂治疗。80 例（52.6%）患儿有损伤，平均 SDI 评分为 1.3±1.7。损伤主要发生在生长（26.8%）、肾脏（17.1%）和神经精神（15.8%）领域。由于进行性肾病，14 例患者需要透析，其中 5 例接受了肾移植。有 9 例与 SLE 相关的死亡，其中 8 例死于感染。基于逻辑回归分析，患者疾病损伤与发病年龄小、疾病持续时间长显著相关。同样，与 SLE 相关的死亡受发病早的影响。相反，性别、疾病持续时间和治疗均未影响所提出的结局指标。
我们的结果与其他三级中心的报告相似。发病早可能会影响儿童 SLE 的长期结局。感染仍然是儿童 SLE 死亡的重要原因。
