Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Eaton Road, Liverpool L12 2AP, UK.
Pediatr Nephrol. 2013 Mar;28(3):363-74. doi: 10.1007/s00467-012-2184-y. Epub 2012 May 16.
Over 80 % of patients with juvenile-onset systemic lupus erythematosus will have renal involvement compared to 40 % with adult-onset disease. Up to 44 % of children who do have lupus nephritis (LN) progress to renal failure in early adulthood. Improved methods of detecting onset of LN would allow earlier treatment, which may prevent irreversible renal scarring and a decline in renal function. Current conventional markers of disease activity fail to adequately predict renal lupus flares and include proteinuria, complement levels, anti-double-stranded DNA antibodies and serum creatinine concentrations. Standardized histological classification is currently the gold standard for diagnosing and classifying LN, but its invasive nature limits routine use for monitoring, especially in a childhood population. Novel biomarkers need to be sensitive and specific-and preferably non-invasive and cost-effective. The most promising biomarkers in juvenile-onset LN include urinary neutrophil gelatinase associated lipocalin, monocyte chemoattractant protein 1 and transforming growth factor-beta, although many others have been identified and are under investigation. No one biomarker yet discovered is unique to LN, indicating an overlap in disease pathophysiology. It is likely that a combination of biomarkers will be required for assessing disease flare detection, response to treatment and prognostic information. Potential biomarkers require longitudinal validation in large paediatric, prospective cohorts to assess their ability to act as clinically useful adjuncts.
与成人发病的系统性红斑狼疮相比，80%以上的青少年发病系统性红斑狼疮患者会出现肾脏受累。多达 44%的狼疮肾炎（LN）患儿会在成年早期进展为肾衰竭。改进 LN 发病的检测方法可以进行更早的治疗，这可能防止不可逆转的肾脏瘢痕和肾功能下降。目前，疾病活动的常规标志物不能充分预测狼疮肾炎的发作，包括蛋白尿、补体水平、抗双链 DNA 抗体和血清肌酐浓度。标准化的组织学分类目前是诊断和分类 LN 的金标准，但它的侵袭性限制了其常规用于监测的用途，尤其是在儿童人群中。新的生物标志物需要具有敏感性和特异性，最好是非侵入性和具有成本效益。在青少年发病的 LN 中，最有前途的生物标志物包括尿中性粒细胞明胶酶相关脂质运载蛋白、单核细胞趋化蛋白 1 和转化生长因子-β，尽管已经确定了许多其他标志物并正在进行研究。尚未发现一种生物标志物是 LN 所特有的，这表明疾病病理生理学存在重叠。很可能需要结合多种生物标志物来评估疾病发作的检测、治疗反应和预后信息。潜在的生物标志物需要在大型儿科前瞻性队列中进行纵向验证，以评估它们作为临床有用的辅助手段的能力。
