Department of Pediatric Rheumatology, Faculty of Medicine, Cukurova University, Adana, Turkey.
Department of Pediatric Nephrology, Faculty of Medicine, Cukurova University, Adana, Turkey.
Clin Rheumatol. 2019 May;38(5):1459-1468. doi: 10.1007/s10067-019-04433-4. Epub 2019 Jan 16.
This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey.
Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2005 and June 2018 were included in the present study.
The median age at the diagnosis was 12.8 (range, 5.1-17.7) years. The female to male ratio was 9.6:1. The most prevalent clinical features were mucocutaneous involvement (96.2%) and constitutional manifestations (94.3%). Renal manifestations, hematological manifestations, and neuropsychiatric involvement were detected in 40 (75%), in 38 (71.7%), and in 13 (24.5%) patients, respectively. Renal biopsy was performed to 49 patients (92.5%). Class IV lupus nephritis (LN) (34%) and class II LN (20.4%) were the most common findings. Mycophenolate mofetil, cyclophosphamide with corticosteroid were the main treatment options. Eighteen patients received rituximab and one tocilizumab. The mean SLE Disease Activity Index (SLEDAI) score at the time of diagnosis was 22.47 ± 8.8 (range = 3-49), and 1.34 ± 1.85 (range = 0-7) at last visit. Twenty-one patients (39.6%) had damage in agreement with Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (PedSDI; mean = 0.60 ± 0.94; range = 0-5) criteria. Growth failure was the most prevalent cause of damage (n = 13, 26%). One patient deceased due to severe pulmonary hemorrhage and multiple cerebral thromboses.
jSLE patients in this cohort have severe disease in view of the higher frequency of renal and neurologic involvement. Nevertheless, multicenter studies are needed to make a conclusion for all Turkish children with jSLE.
本研究旨在分析来自土耳其南部的幼年系统性红斑狼疮（jSLE）患者的临床特征、实验室数据、疾病活动度和转归。
本研究纳入了 2005 年 1 月至 2018 年 6 月期间根据修订后的美国风湿病学会 1997 年标准诊断为 jSLE 的 53 例患者。
诊断时的中位年龄为 12.8 岁（范围：5.1-17.7 岁）。女性与男性的比例为 9.6:1。最常见的临床特征为黏膜皮肤受累（96.2%）和全身表现（94.3%）。40 例（75%）患者出现肾脏表现、38 例（71.7%）患者出现血液学表现、13 例（24.5%）患者出现神经精神表现。对 49 例（92.5%）患者进行了肾脏活检。最常见的发现是Ⅳ型狼疮肾炎（34%）和Ⅱ型狼疮肾炎（20.4%）。霉酚酸酯、环磷酰胺联合皮质类固醇是主要的治疗选择。18 例患者接受了利妥昔单抗治疗，1 例患者接受了托珠单抗治疗。诊断时平均 SLE 疾病活动指数（SLEDAI）评分为 22.47±8.8（范围：3-49），末次就诊时为 1.34±1.85（范围：0-7）。21 例（39.6%）患者符合系统性红斑狼疮国际合作临床/美国风湿病学会损害指数（PedSDI）的损害标准（平均 0.60±0.94；范围 0-5）。生长发育迟缓是最常见的损害原因（n=13，26%）。1 例患者因严重肺出血和多发脑梗死死亡。
本队列中的 jSLE 患者肾脏和神经受累频率较高，提示疾病严重。然而，需要进行多中心研究才能得出关于所有土耳其 jSLE 儿童的结论。
