Miguel Daniele Faria, Terreri Maria Teresa, Pereira Rosa Maria Rodrigues, Bonfá Eloisa, Silva Clovis Artur Almeida, Corrente José Eduardo, Magalhaes Claudia Saad
Universidade Estadual Paulista (UNESP) Faculdade de Medicina de Botucatu, Botucatu, Brazil.
Pediatric Rheumatology Division, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), São Paulo, Brazil.
Adv Rheumatol. 2020 Feb 1;60(1):10. doi: 10.1186/s42358-020-0114-4.
Urinary parameters, anti-dsDNA antibodies and complement tests were explored in patients with childhood-Systemic Lupus Erythematosus (cSLE) early-onset lupus nephritis (ELN) from a large multicenter cohort study.
Clinical and laboratory features of cSLE cases with kidney involvement at presentation, were reviewed. Disease activity parameters including SLEDAI-2 K scores and major organ involvement at onset and follow up, with accrued damage scored by SLICC-DI, during last follow up, were compared with those without kidney involvement. Autoantibodies, renal function and complement tests were determined by standard methods. Subjects were grouped by presence or absence of ELN.
Out of the 846 subjects enrolled, mean age 11.6 (SD 3.6) years; 427 (50.5%) had ELN. There was no significant difference in the ELN proportion, according to onset age, but ELN frequency was significantly higher in non-Caucasians (p = 0.03). Hematuria, pyuria, urine casts, 24-h proteinuria and arterial hypertension at baseline, all had significant association with ELN outcome (p < 0.001). With a similar follow up time, there were significantly higher SLICC-DI damage scores during last follow up visit (p = 0.004) and also higher death rates (p < 0.0001) in those with ELN. Low C3 (chi-square test, p = 0.01), but not C3 levels associated significantly with ELN. High anti-dsDNA antibody levels were associated with ELN (p < 0.0001), but anti-Sm, anti-RNP, anti-Ro, anti-La antibodies were not associated. Low C4, C4 levels, low CH50 and CH50 values had no significant association. High erythrocyte sedimentation rate (ESR) was associated with the absence of ELN (p = 0.02).
The frequency of ELN was 50%, resulting in higher morbidity and mortality compared to those without ELN. The urinary parameters, positive anti-dsDNA and low C3 are reliable for discriminating ELN.
在一项大型多中心队列研究中,对儿童系统性红斑狼疮(cSLE)早发性狼疮性肾炎(ELN)患者的尿液参数、抗双链DNA抗体和补体检测进行了探索。
回顾了初诊时伴有肾脏受累的cSLE病例的临床和实验室特征。将疾病活动参数(包括SLEDAI-2K评分)、发病时和随访时的主要器官受累情况,以及最后一次随访时根据SLICC-DI计算的累积损伤评分,与无肾脏受累的患者进行比较。通过标准方法测定自身抗体、肾功能和补体检测。根据是否存在ELN对受试者进行分组。
在纳入的846名受试者中,平均年龄11.6岁(标准差3.6岁);427名(50.5%)患有ELN。ELN比例按发病年龄无显著差异,但非白种人中ELN的发生率显著更高(p = 0.03)。基线时的血尿、脓尿、尿沉渣、24小时蛋白尿和动脉高血压,均与ELN结局有显著关联(p < 0.001)。在相似的随访时间内,ELN患者在最后一次随访时的SLICC-DI损伤评分显著更高(p = 0.004),死亡率也更高(p < 0.0001)。低C3(卡方检验,p = 0.01),但C3水平与ELN无显著关联。高抗双链DNA抗体水平与ELN相关(p < 0.0001),但抗Sm、抗RNP、抗Ro、抗La抗体与之无关。低C4、C4水平、低CH50和CH50值无显著关联。高红细胞沉降率(ESR)与无ELN相关(p = 0.02)。
ELN的发生率为50%,与无ELN的患者相比,其发病率和死亡率更高。尿液参数、抗双链DNA阳性和低C3对鉴别ELN可靠。