Division of Pediatric Nephrology, University of Miami/Holtz Children's Hospital, Miami, FL 33101, USA.
Clin J Am Soc Nephrol. 2011 Sep;6(9):2192-9. doi: 10.2215/CJN.00910111. Epub 2011 Jul 28.
Childhood-onset lupus nephritis (LN) carries a worse renal prognosis compared with adults. Controlled treatment trials in children are lacking. We compared renal and patient survival in a cohort of pediatric patients followed over 3 decades.
DESIGN, SETTINGS, PARTICIPANTS, & MEASUREMENTS: A retrospective analysis was conducted on 138 patients with childhood-onset systemic lupus erythematosus from 1980 to 2010. The core cohort included 95 with severe LN: 28 progressed to end-stage renal disease (ESRD group) whereas 67 did not (no-ESRD group). Patients were stratified into four "eras" according to the introduction of the primary immuno-suppressive drug: era 1: triple oral therapy with corticosteroids (CS), cyclophosphamide (CYC), and azathioprine (AZA); era 2: intravenous CYC; era 3: mycophenolate mofetil (MMF) ± CYC; era 4: rituximab (RTX) ± CYC ± MMF.
Mean age at diagnosis was 12.3 ± 2.9 years with median follow-up of 5 years. Poor renal function (estimated GFR < 60 ml/min per 1.73 m(2)) and nephrotic proteinuria at diagnosis imparted a poor prognosis. Increasing proteinuria correlated with progression of kidney disease. The addition of MMF in era 3 improved 5-year renal survival from 52% to 91% and overall patient survival from 83% to 97%. African-American ethnicity was associated with significant risk for progression to ESRD whereas Hispanic ethnicity conferred an advantage. Infection and cardiovascular disease were the primary causes of patient demise.
Renal and patient survival in childhood-onset LN has improved during the past 3 decades with progressive treatment regimens. Future trials in children are very much warranted.
与成人相比，儿童发病的狼疮性肾炎（LN）具有更差的肾脏预后。缺乏对儿童的对照治疗试验。我们对过去 30 多年来随访的一组儿科患者进行了比较，以比较其肾脏和患者生存率。
设计、地点、参与者和测量方法：对 1980 年至 2010 年间的 138 例儿童发病系统性红斑狼疮患者进行了回顾性分析。核心队列包括 95 例严重 LN 患者：28 例进展为终末期肾病（ESRD 组），而 67 例未进展（非 ESRD 组）。根据主要免疫抑制剂药物的引入，患者分为四个“时代”：时代 1：皮质类固醇（CS）、环磷酰胺（CYC）和硫唑嘌呤（AZA）三联口服治疗；时代 2：静脉内 CYC；时代 3：霉酚酸酯（MMF）±CYC；时代 4：利妥昔单抗（RTX）±CYC±MMF。
诊断时的平均年龄为 12.3±2.9 岁，中位随访时间为 5 年。诊断时肾功能不佳（估计肾小球滤过率＜60ml/min/1.73m2）和肾病性蛋白尿提示预后不良。蛋白尿的增加与肾脏疾病的进展相关。在时代 3 中添加 MMF 将 5 年的肾脏生存率从 52%提高到 91%，总患者生存率从 83%提高到 97%。非裔美国人的种族与进展为 ESRD 的显著风险相关，而西班牙裔则具有优势。感染和心血管疾病是患者死亡的主要原因。
在过去的 30 年中，随着治疗方案的不断发展，儿童发病的 LN 的肾脏和患者生存率得到了提高。非常需要在儿童中进行未来的试验。
