伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析

目的 分析伴抗中性粒细胞胞浆抗体（ANCA）阳性狼疮性肾炎（LN）患儿的临床及病理特征。方法 回顾性选择2003年1月至2019年12月于中山大学附属第一医院诊断的LN患儿，依据血清ANCA结果分为ANCA阳性组（n=59）和ANCA阴性组（n=454），比较两组患儿临床表现、组织病理学、治疗缓解与预后情况。结果 ANCA阳性组白细胞降低并伴红细胞沉降率增快（P < 0.05）。两组患儿血肌酐、尿蛋白、尿红细胞差异无统计学意义（P > 0.05）。308例患儿行肾脏穿刺活检，光镜结果提示ANCA阳性组细胞纤维性新月体比例高于ANCA阴性组（P < 0.05），免疫复合物沉积比例低于ANCA阴性组（P < 0.05）。两组患儿治疗缓解率、生存情况差异无统计学意义（P > 0.05）。结论 ANCA阳性LN患儿肾脏病理损害更重，与临床表现不完全对应平行，及时行肾脏穿刺活检至关重要。

Clinicopathological features of children with lupus nephritis with positive antineutrophil cytoplasmic antibody

Objective To study the clinicopathological features of children with lupus nephritis (LN) with positive anti-neutrophil cytoplasmic antibody (ANCA). Methods A retrospective analysis was performed for the children who were diagnosed with LN in the First Affiliated Hospital of Sun Yat-sen University from January 2003 to December 2019. According to the results of serum ANCA, they were divided into two groups: ANCA-positive group (n=59) and ANCAnegative group (n=454). The two groups were compared in terms of clinical manifestations, histopathological features, remission rate, and prognosis. Results Compared with the ANCA-negative group, the ANCA-positive group had a significant reduction in leukocytes and a significant increase in erythrocyte sedimentation rate (P < 0.05). There were no significant differences between the two groups in serum creatinine, urine protein, and urine red blood cell count (P > 0.05). A total of 308 children underwent kidney biopsy. The results on light microscopy showed that compared with the ANCAnegative group, the ANCA-positive group had a significantly higher proportion of children with cellular fibrous crescents (P < 0.05) and a significantly lower proportion of children with immune complex deposition (P < 0.05). There were no significant differences between the two groups in the remission rate and survival rate (P > 0.05). Conclusions Children with ANCA-positive LN tend to have more severe renal pathological injury, which is not exactly parallel with clinical manifestations, suggesting that timely renal biopsy is of great importance.