目的 分析核周型抗中性粒细胞胞浆抗体（pANCA）检测对儿童系统性红斑狼疮（SLE）的临床意义。方法 回顾性分析2016年1月至2021年7月就诊的SLE患儿的临床资料,对pANCA阳性和阴性患儿的自身抗体相关指标、免疫功能、肾功能及肾脏病理进行分析。结果 共纳入191例SLE患儿,女160例、男31例,平均年龄（12.0±3.5）岁。pANCA阳性组81例,其中pANCA（甲醛抗性）阳性26例、pANCA（甲醛敏感）阳性55例;抗髓过氧化物酶（MPO）抗体阳性29例,占pANCA阳性患儿的35.8%。与pANCA阴性组相比,pANCA阳性组患儿血沉较高,Hb较低,自身抗体中抗核抗体、抗双链DNA（dsDNA）抗体、抗干燥综合征抗原 A（SSA）抗体、抗着丝点蛋白B（CENP-B）抗体、抗组蛋白抗体、抗核糖体核蛋白（rRNP）抗体、抗核小体抗体以及抗Ro-52抗体的阳性率均较高,差异有统计学意义（P<0.05）。与pANCA阴性组相比,pANCA阳性组患儿补体C3和C4较低,尿肌酐、尿免疫球蛋白G/尿肌酐（UCr）、α1-微球蛋白/UCr、β1-微球蛋白/UCr、尿微量白蛋白/UCr水平较高,尿隐血和尿蛋白比例较高,差异均有统计学意义（P<0.05）。共112例SLE患儿行肾穿刺活检,与pANCA阴性组相比,pANCA阳性组系膜增生比例较高,肾小球系膜和毛细血管袢的IgG、IgM、IgA、C1q和C3沉积比例较高,差异均有统计学意义（P<0.05）。结论 在儿童SLE中,pANCA阳性提示更容易发生肾损伤,病情更严重。

Objective To investigate the clinical significance of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) in children with systemic lupus erythematosus. Methods The clinical data of SLE children treated from January 2016 to July 2021 were retrospectively analyzed. The autoantibody related indexes, immune function, renal function and renal pathology of pANCA positive and negative children were analyzed. Results A total of 191 SLE children (160 girls and 31 boys) were enrolled, and the average age was (12.0±3.5) years. There were 81 children in pANCA positive group, among whom 26 were formaldehyde-resistant pANCA positive and 55 were formaldehyde-sensitive pANCA positive. Anti-myeloperoxidase (MPO) antibody was positive in 29 children, accounting for 35.8% of pANCA positive children. Compared with pANCA negative group, children in pANCA positive group had higher ESR, lower Hb, and higher positive rates of antinuclear antibody, anti-double-stranded DNA (dsDNA) antibody, anti-Sjögren's-syndrome-related antigen A (SSA) autoantibody, anticentromere protein B (CENP-B) antibody, anti-histone antibody, anti-ribosome ribonucleoprotein (rRNP) antibody, anti-nucleosome antibody and anti-Ro-52 antibody. The differences were statistically significant (P<0.05). Compared with pANCA negative group, pANCA positive group had lower levels of complement C3 and C4, higher levels of urinary creatinine (UCr), urinary immunoglobulin G/UCr, α1-microglobulin/UCr, β1-microglobulin/UCr and urinary microalbumin/UCr, and higher proportion of urinary occult blood and urinary protein, and the differences were statistically significant (P<0.05). A total of 112 children with SLE underwent renal biopsy. Compared with the pANCA negative group, the proportion of mesangial hyperplasia and the deposition proportion of IgG, IgM, IgA, C1q and C3 in glomerular mesangium and capillary loop was higher in the pANCA positive group, and the differences were statistically significant (P<0.05). Conclusions In children with SLE, pANCA positive children are more likely to have renal injury and more severe symptoms.