Department of Pediatrics, Faculty of Medicine, Cairo University, Giza, Giza, Egypt.
Rheumatol Int. 2012 Jan;32(1):47-51. doi: 10.1007/s00296-010-1554-7. Epub 2010 Jul 24.
Lupus nephritis has been described as the most serious complication of systemic lupus erythematosus (SLE) and the strongest predictor of poor outcome. While the incidence of childhood SLE is relatively low, renal involvement appears to be more common and more severe in childhood SLE. This study aims to characterize the features and outcome of renal involvement in childhood-onset SLE based on a study of 100 Egyptian patients (mean age at diagnosis 10.1 years, range 2-17 years). Initial data regarding disease manifestations and biopsy findings were reviewed. Disease activity was assessed using SLEDAI scores. Follow-up data (mean duration 6 years) were noted regarding specific treatment, response, complications and renal survival. Initial renal involvement was present in 78 patients, including 66 with hypertension and 23 with renal impairment. Pathologically, class IV nephropathy was found in 18 patients, class V in 9 and low-grade lesions (class II-III) in 49. Twenty patients required follow-up biopsy, and all transformations were observed. SLEDAI scores significantly decreased from initial (mean ± SD) of 21.4 ± 7.3 to 13.4 ± 7.8, in association with response to therapy (P < 0.0001). Poor response was associated with initial hypertension and renal impairment but not with initial SLEDAI score or pathological class. The projected renal survival was 82.4 and 64.7% 5 and 10 years from diagnosis. Early renal involvement in childhood SLE is common, serious and requires proper evaluation and management.
狼疮肾炎是系统性红斑狼疮（SLE）最严重的并发症，也是预后不良的最强预测因素。虽然儿童 SLE 的发病率相对较低，但肾脏受累在儿童 SLE 中似乎更为常见且更为严重。本研究旨在根据对 100 例埃及患者（诊断时的平均年龄为 10.1 岁，范围为 2-17 岁）的研究，描述儿童发病的狼疮肾炎的特征和结局。回顾了疾病表现和活检结果的初始数据。使用 SLEDAI 评分评估疾病活动度。记录了关于特定治疗、反应、并发症和肾脏存活率的随访数据（平均随访时间为 6 年）。78 例患者存在初始肾脏受累，包括 66 例高血压和 23 例肾功能不全。18 例患者存在病理 IV 级肾病，9 例存在病理 V 级肾病，49 例存在低级别病变（病理 II-III 级）。20 例患者需要进行随访活检，所有患者均发生了病变转化。SLEDAI 评分从初始（平均值±标准差：21.4±7.3）显著降低至 13.4±7.8，与治疗反应相关（P＜0.0001）。治疗反应不佳与初始高血压和肾功能不全相关，与初始 SLEDAI 评分或病理分级无关。诊断后 5 年和 10 年的预计肾脏存活率分别为 82.4%和 64.7%。儿童 SLE 早期的肾脏受累常见且严重，需要进行适当的评估和管理。
