Gau Chun-Chun, Tseng Min-Hua, Wu Chao-Yi, Yang Huang-Yu, Huang Jing-Long
Division of Allergy, Asthma, and Rheumatology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan.
Division of Pediatric General Medicine, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan.
Front Med (Lausanne). 2021 Apr 16;8:647510. doi: 10.3389/fmed.2021.647510. eCollection 2021.
Systemic lupus erythematosus (SLE), an autoimmune disease, is characterized by the overproduction of autoantibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) have been recognized in SLE for decades. To date, their association with SLE disease activity, especially in pediatric-onset SLE (pSLE) patients, is limited. We conducted a retrospective case-control study of pSLE patients with ANCAs from 2010 to 2020. Clinical characteristics, laboratory data, renal histological features, treatment and outcomes were analyzed. A total of 70 pediatric-onset SLE patients (9 ANCA-positive vs. 61 ANCA-negative) with a median age of 12.23 years (age ranging from 4 years to 18 years) at diagnosis were enrolled. Among patients with ANCAs, MPO-ANCA was found in seven and PR3-ANCA in two of those cases. Patients with ANCAs had a tendency to have hematuria compared with those without ANCAs (66 vs. 24.6%, respectively; = 0.026). Of the 70 SLE patients, 8 with ANCAs and 44 without ANCAs underwent renal biopsies. Patients with ANCAs (25%, 2/8) were more likely to lack the typical full-house pattern in their renal immunofluorescence (IF) staining. pSLE patients with ANCAs tend to have hematuria and an absence of typical IF histology. However, patients with and without ANCAs showed no difference in their clinical presentations and treatment outcomes.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，其特征是自身抗体过度产生。抗中性粒细胞胞浆抗体（ANCA）在SLE中已被认识数十年。迄今为止，它们与SLE疾病活动的关联，尤其是在儿童期发病的SLE（pSLE）患者中，是有限的。我们对2010年至2020年患有ANCA的pSLE患者进行了一项回顾性病例对照研究。分析了临床特征、实验室数据、肾脏组织学特征、治疗及结局。共纳入70例儿童期发病的SLE患者（9例ANCA阳性与61例ANCA阴性），诊断时中位年龄为12.23岁（年龄范围4岁至18岁）。在ANCA阳性患者中，7例发现髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA），2例发现蛋白酶3抗中性粒细胞胞浆抗体（PR3-ANCA）。与无ANCA的患者相比，有ANCA的患者有血尿倾向（分别为66%和24.6%；P = 0.026）。在70例SLE患者中，8例有ANCA的患者和44例无ANCA的患者接受了肾脏活检。有ANCA的患者（25%，2/8）在肾脏免疫荧光（IF）染色中更易缺乏典型的满堂亮模式。有ANCA的pSLE患者倾向于有血尿且缺乏典型的IF组织学表现。然而，有和无ANCA的患者在临床表现和治疗结局方面无差异。
