Clinical characteristics of children with anti-neutrophil cytoplasmic antibody-associated vasculitis
10.7499/j.issn.1008-8830.2401074
- VernacularTitle:儿童抗中性粒细胞胞质抗体相关性血管炎临床特征分析
- Author:
Jing-Qi LIU
1
;
Yong-Zhen LI
;
Lan-Jun SHUAI
;
Yan CAO
;
Xiao-Yan LI
;
Ying WANG
;
Tian SHEN
;
Shuang-Hong MO
;
Xiao-Jie HE
;
Xiao-Chuan WU
Author Information
1. 中南大学湘雅二医院儿童医学中心,湖南长沙 410011
- Keywords:
Anti-neutrophil cytoplasmic antibody-associated vasculitis;
Clinical manifestation;
Chronic kidney disease;
Prognosis;
Child
- From:
Chinese Journal of Contemporary Pediatrics
2024;26(8):823-828
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinical characteristics of children with anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).Methods A retrospective analysis was conducted on the clinical data of 25 children diagnosed with AAV at the Second Xiangya Hospital of Central South University from January 2010 to June 2022.Results Among the AAV children,there were 5 males and 20 females,with a median age of onset of 11.0 years.Involvement of the urinary system was seen in 18 cases(72%);respiratory system involvement in 10 cases(40%);skin involvement in 6 cases(24%);eye,ear,and nose involvement in 5 cases(20%);joint involvement in 4 cases(16%);digestive system involvement in 2 cases(8%).Eleven cases underwent kidney biopsy,with 5 cases(46%)showing focal type,2 cases(18%)showing crescentic type,2 cases(18%)showing mixed type,and 2 cases(18%)showing sclerotic type.Immune complex deposits were present in 5 cases(45%).Seven cases reached chronic kidney disease(CKD)stage Ⅴ,with 2 cases resulting in death.Two cases underwent kidney transplantation.At the end of the follow-up period,2 cases were at CKD stage Ⅱ,and 1 case was at CKD stage Ⅲ.Of the 16 cases of microscopic polyangiitis(MPA)group,13(81%)involved the urinary system.Of the 9 cases of granulomatosis with polyangiitis(GPA),6 cases(66%)had sinusitis.Serum creatinine and uric acid levels were higher in the MPA group than in the GPA group(P<0.05),while red blood cell count and glomerular filtration rate were lower in the MPA group(P<0.05).Conclusions AAV is more common in school-age female children,with MPA being the most common clinical subtype.The onset of AAV in children is mainly characterized by renal involvement,followed by respiratory system involvement.The renal pathology often presents as focal type with possible immune complex deposits.Children with MPA often have renal involvement,while those with GPA commonly have sinusitis.The prognosis of children with AAV is poor,often accompanied by renal insufficiency.
