Jin Si-Yan, Huang Dan-Lin, Dang Xi-Qiang, Yi Zhu-Wen
a Division of Paediatric Nephrology, Department of Paediatrics , Central South University , Changsha , P. R. China.
Paediatr Int Child Health. 2017 Nov;37(4):286-291. doi: 10.1080/20469047.2017.1309337. Epub 2017 May 2.
System lupus erythematosus (SLE) is a severe multisystem autoimmune disease.
To describe the clinical and pathological features, treatment, and renal outcome in children under 18 years with lupus nephritis (LN).
The study was undertaken by a questionnaire completed in 26 Grade 3A hospitals' paediatric renal units in China. The study comprised 788 children (619 girls, 169 boys) diagnosed with SLE by the American College of Rheumatology criteria (1997) during 2005-2010. Results of renal biopsies were classified according to the guidelines of The International Association of Nephrology and the Renal Pathology Society (2003). Guidelines by the Chinese Society of Paediatric Nephrology were applied for the diagnosis and treatment (for trial implementation) in 2010 to determine inclusion. The data included the prevalence of acute kidney injury (AKI), SLE disease activity index (SLEDAI), renal histopathology and the induction of therapy mode.
The mean (SD) age of onset of SLE was 10.9 (2.90) years (range 1-18) and at diagnosis was 11.3 (2.9) years. The mean (SD) SLEDAI score was 13.5 (5.53). The clinical classification was as follows: about 36 (4.6%) patients had isolated haematuria, 99 (12.6%) isolated proteinuria, 60 (7.6%) isolated haematuria and proteinuria, 157 (19.9%) acute glomerulonephritis, 392 (49.7%) nephrotic syndrome, 20 (2.5%) rapidly progressive glomerulonephritis, 15 (1.9%) chronic nephritis, 2 (0.3%) tubule-interstitial damage and 7 (0.9%) subclinical LN. A total of 549 children (69.7%) underwent renal biopsy. The most frequent renal histopathological findings of LN were Class IV, followed by Class II and Class V + IV. There were no significant differences between the age groups in either renal pathological types or prognosis. In 242 (30.7%) patients, LN was complicated by AKI. Those with AKI had an older mean (SD) age at onset than the non-AKI patients [11.5 (2.8) years vs 10.7 (2.9) years, respectively, p < 0.0001] and a higher SLEDAI score [14.3 (5.8) vs 13.1 (5.4), respectively, p = 0.003]. In the induction phase, cyclophosphamide (CTX) and mycophenolate mofetil (MMF) were equally effective in the patients with the same pathological type. Follow-up records were only available for 482 (61.2%) patients, with a mean (SD) follow-up time of 21.5 (18.4) months. Six of the 35 patients who deteriorated required dialysis and seven died.
In LN, AKI is a risk factor for poor outcome. Owing to different times of onset and remission, the pathological types of LN cannot be estimated by clinical manifestation alone, and therefore renal biopsy should be undertaken in all LN children with AKI. In the induction phase, there was no significant difference in efficacy between CTX and MMF. Follow-up of children with LN in China needs to be improved.
系统性红斑狼疮（SLE）是一种严重的多系统自身免疫性疾病。
描述18岁以下狼疮性肾炎（LN）患儿的临床和病理特征、治疗及肾脏转归。
本研究通过对中国26家三级甲等医院儿科肾脏单位填写的问卷进行。该研究纳入了2005年至2010年间根据美国风湿病学会标准（1997年）诊断为SLE的788例儿童（619例女孩，169例男孩）。肾活检结果根据国际肾脏病学会和肾脏病理学会指南（2003年）进行分类。2010年应用中华儿科肾脏病学会的指南进行诊断和治疗（试行）以确定纳入标准。数据包括急性肾损伤（AKI）的发生率、SLE疾病活动指数（SLEDAI）、肾脏组织病理学及诱导治疗方式。
SLE的平均（标准差）发病年龄为10.9（2.90）岁（范围1 - 18岁），诊断时年龄为11.3（2.9）岁。SLEDAI平均（标准差）评分为13.5（5.53）。临床分类如下：约36例（4.6%）患者为单纯血尿，99例（12.6%）为单纯蛋白尿，60例（7.6%）为血尿和蛋白尿，157例（19.9%）为急性肾小球肾炎，392例（49.7%）为肾病综合征，20例（2.5%）为急进性肾小球肾炎，15例（1.9%）为慢性肾炎，2例（0.3%）为肾小管间质损害，7例（0.9%）为亚临床LN。共有549例儿童（69.7%）接受了肾活检。LN最常见的肾脏组织病理学表现为IV型，其次为II型和V + IV型。各年龄组在肾脏病理类型或预后方面无显著差异。242例（30.7%）患者的LN合并AKI。合并AKI的患者发病时的平均（标准差）年龄大于未合并AKI的患者[分别为11.5（2.8）岁和10.7（2.9）岁，p < 0.0001]，且SLEDAI评分更高[分别为14.3（5.8）和13.1（5.4），p = 0.003]。在诱导期，环磷酰胺（CTX）和霉酚酸酯（MMF）对相同病理类型的患者疗效相当。仅482例（61.2%）患者有随访记录，平均（标准差）随访时间为21.5（18.4）个月。35例病情恶化的患者中有6例需要透析，7例死亡。
在LN中，AKI是预后不良的危险因素。由于发病和缓解时间不同，LN的病理类型不能仅通过临床表现来估计，因此所有合并AKI的LN患儿均应进行肾活检。在诱导期，CTX和MMF的疗效无显著差异。中国LN患儿的随访工作需要改进。
