Department of Pediatrics, Hebei Medical University, Shijiazhuang, China.
Department of Pediatric Nephrology and Immunology, Children's Hospital of Hebei Province, Shijiazhuang, China.
BMC Nephrol. 2022 Aug 26;23(1):296. doi: 10.1186/s12882-022-02921-5.
Endocapillary hypercellularity (ECHC) is commonly seen in class IV lupus nephritis (LN), the most common and severe LN in children. Factors influencing early complete remission (CR) in pediatric class IV LN have been poorly described. We investigated the relationship between ECHC levels and early CR in pediatric class IV LN.
Patients with newly, simultaneously diagnosed systemic lupus erythematosus (SLE) and class IV LN by renal biopsy from 2012 to 2021 were studied. In this retrospective study, two pathologists who were blind to clinical information reviewed all pathological data retrospectively and classified glomerular lesions according to the revised criteria of the International Society of Nephrology and the Renal Pathology Society (ISN/RPS). The demographics, baseline clinical characteristics, laboratory parameters, renal histopathological findings, treatment regimen and CR at 6 months after immunosuppressive therapy were analyzed. ECHC was categorized as: > 50% (group A), 25-50% (group B) and < 25% (group C). CR was defined as absence of clinical symptoms, 24-hour urinary protein < 0.15 g, and normal levels of serum creatinine and albumin.
Sixty-four patients were identified: 23, 15 and 26 in groups A, B and C, respectively. Group A had significantly higher levels of D-dimer, urine protein, and SLE disease activity index (SLEDAI) than groups B and C. Group C had a markedly higher estimated glomerular filtration rate (eGFR) than groups A and B. A substantially greater proportion of patients in group A had glomerular microthrombi and basement membrane thickening than in groups B and C. At 6 months post treatment, CR was achieved in 19 (82.6%), 5 (33.3%) and 11 (42.3%) in groups A, B and C, respectively (p < 0.05, group A vs groups B and C). Multiple logistic regression analysis revealed that ECHC and urine protein levels were significantly associated with CR.
ECHC and urine protein levels may be valuable biomarkers for predicting early CR in pediatric class IV LN.
毛细血管内细胞增生(ECHC)常见于儿童中最常见和最严重的 IV 型狼疮肾炎(LN)。影响儿童 IV 型 LN 早期完全缓解(CR)的因素描述较少。我们研究了 ECHC 水平与儿童 IV 型 LN 早期 CR 的关系。
研究了 2012 年至 2021 年期间经肾活检同时诊断为系统性红斑狼疮(SLE)和 IV 型 LN 的患者。在这项回顾性研究中,两名对临床信息不知情的病理学家回顾性地审查了所有病理数据,并根据国际肾脏病学会和肾脏病理学会(ISN/RPS)修订标准对肾小球病变进行分类。分析了人口统计学、基线临床特征、实验室参数、肾脏组织病理学发现、治疗方案以及免疫抑制治疗后 6 个月的 CR。ECHC 分为:>50%(A 组)、25-50%(B 组)和<25%(C 组)。CR 定义为无临床症状、24 小时尿蛋白<0.15g,以及血清肌酐和白蛋白正常。
共确定了 64 例患者:A、B 和 C 组分别为 23、15 和 26 例。A 组的 D-二聚体、尿蛋白和 SLE 疾病活动指数(SLEDAI)水平明显高于 B 和 C 组。C 组的估算肾小球滤过率(eGFR)明显高于 A 和 B 组。A 组有更多的患者肾小球内有微血栓和基底膜增厚,B 和 C 组则较少。治疗后 6 个月,A、B 和 C 组的 CR 分别为 19(82.6%)、5(33.3%)和 11(42.3%)(p<0.05,A 组与 B 和 C 组相比)。多因素逻辑回归分析显示,ECHC 和尿蛋白水平与 CR 显著相关。
ECHC 和尿蛋白水平可能是预测儿童 IV 型 LN 早期 CR 的有价值的生物标志物。