| 摘要: |
| 目的:探讨儿童抗中性粒细胞胞质抗体相关性血管炎(AAV)的临床特点。方法:回顾性分析2017 年1 月至2024 年2 月
河南省儿童医院郑州儿童医院收治的14 例儿童AAV 患者的临床资料。结果:14 例患儿中,男3 例,女11 例,中位年龄9 岁,其
中显微镜下多血管炎(MPA)13 例,肉芽肿性多血管炎(GPA)1 例。14 例患儿中12 例出现肾脏受累,3 例行肾脏病理检查,均符
合寡免疫坏死性新月体肾炎;11 例患儿出现了肺受累,其中5 例进展为呼吸衰竭,4 例出现肺出血;12 例贫血。14 例患儿均应
用糖皮质激素,其中10 例联合应用环磷酰胺冲击治疗,2 例应用利妥昔单抗治疗,2 例应用吗替麦考酚酯,1 例应用环孢素;9 例
行血浆置换治疗,1 例行血液灌流治疗;8 例肾功能衰竭患儿行血液透析,其中4 例后续行腹膜透析治疗;经治疗4 例达临床缓
解,尿检及肾功能正常;3 例肾功能衰竭患儿行肾移植;3 例死亡;3 例儿童失访;1 例持续腹膜透析中。结论:儿童AAV 临床发
病率低且临床表现复杂多样,病死率高,肺和肾脏是其主要靶器官,临床易漏诊、误诊,应尽早诊断及时实施个体化规范治疗。 |
| 关键词: 儿童 抗中性粒细胞胞质抗体相关性血管炎 抗中性粒细胞胞质抗体相关性肾小球肾炎 |
| DOI：doi:10.13407/j.cnki.jpp.1672-108X.2025.07.004 |
|
| 基金项目:2023 年度河南省医学科技攻关计划联合共建项目,编号LHGJ20230603 |
|
| Cross-Sectional Study on Children with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis |
| Li Yuliu, Liu Yujie, Cao Guanghai, Tian Ming, Liu Cuihua |
| ((Children’s Hospital Affiliated to Zhengzhou University, Henan
Children’s Hospital, Zhengzhou Children’s Hospital, Zhengzhou 450018, China)) |
| Abstract: |
| Objective: To explore the clinical characteristics of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) in
children. Methods: Clinical data of 14 children with AAV admitted into Henan Children’s Hospital, Zhengzhou Children’s Hospital
from Jan. 2017 to Feb. 2024 were retrospectively analyzed. Results: Among the 14 children, there were 3 boys and 11 girls, with a
median age of 9 years. Among them, there were 13 cases of microscopic polyangiitis (MPA) and 1 case of granulomatosis with
polyangiitis (GPA). Among the 14 children, 12 cases had renal involvement, 3 cases underwent kidney biopsy, and all of them were
consistent with pauci-immune necrotizing crescentic glomerulonephritis. Lung involvement occurred in 11 children, 5 children progressed
to respiratory failure and 4 children had pulmonary hemorrhage. And 12 cases had anemia. All children were treated with
glucocorticoids, 10 cases were treated with cyclophosphamide pulse therapy, 2 cases were given rituximab, 2 cases were given
mycophenolate mofetil, and 1 case received cyclosporine. Nine cases underwent plasma exchange, and 1 case was given hemoperfusion
treatment. Eight children with renal failure underwent hemodialysis, among whom 4 cases subsequently received peritoneal dialysis
treatment. After treatment, 4 cases achieved clinical remission with normal urine test and renal function. Renal transplantation was
performed in 3 children with renal failure, 3 children died, 3 children were lost to follow-up, 1 child was on continuous peritoneal
dialysis. Conclusion: The clinical incidence of AAV in children is low, and the clinical manifestations are complex and diverse with a
high fatality rate. The lung and kidney are the main target organs of AAV, and the probability of clinical missed diagnosis and
misdiagnosis is high. Early clinical diagnosis and individualized standardized treatment should be carried out in clinical practice. |
| Key words: children antineutrophil cytoplasmic antibody-associated vasculitis antineutrophil cytoplasmic antibody-associated
glomerulonephritis |
