Liu Jing-Qi, Li Yong-Zhen, Shuai Lan-Jun, Cao Yan, Li Xiao-Yan, Wang Ying, Shen Tian, Mo Shuang-Hong, He Xiao-Jie, Wu Xiao-Chuan
Children's Medical Center, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2024 Aug 15;26(8):823-828. doi: 10.7499/j.issn.1008-8830.2401074.
To study the clinical characteristics of children with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
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.
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 (<0.05), while red blood cell count and glomerular filtration rate were lower in the MPA group (<0.05).
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.
研究抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）患儿的临床特征。
对2010年1月至2022年6月在中南大学湘雅二医院确诊为AAV的25例患儿的临床资料进行回顾性分析。
AAV患儿中，男性5例，女性20例，中位发病年龄为11.0岁。18例（72%）出现泌尿系统受累；10例（40%）出现呼吸系统受累；6例（24%）出现皮肤受累；5例（20%）出现眼、耳、鼻受累；4例（16%）出现关节受累；2例（8%）出现消化系统受累。11例患儿进行了肾活检，5例（46%）为局灶型，2例（18%）为新月型，2例（18%）为混合型，2例（18%）为硬化型。5例（45%）有免疫复合物沉积。7例达到慢性肾脏病（CKD）5期，2例死亡。2例进行了肾移植。随访结束时，2例处于CKD 2期，1例处于CKD 3期。在显微镜下多血管炎（MPA）组的16例中，13例（81%）累及泌尿系统。在肉芽肿性多血管炎（GPA）组的9例中，6例（66%）有鼻窦炎。MPA组血清肌酐和尿酸水平高于GPA组（<0.05），而MPA组红细胞计数和肾小球滤过率低于GPA组（<0.05）。
AAV在学龄期女童中更常见，MPA是最常见的临床亚型。儿童AAV发病主要以肾脏受累为主，其次为呼吸系统受累。肾脏病理常表现为局灶型，可能有免疫复合物沉积。MPA患儿常累及肾脏，而GPA患儿常见鼻窦炎。儿童AAV预后较差，常伴有肾功能不全。
