目的 探讨抗中性粒细胞胞质抗体（anti-neutrophil cytoplasmic antibody, ANCA）相关性血管炎（ANCA-associated vasculitis, AAV）患儿的临床特征。 方法 回顾性分析中南大学湘雅二医院2010年1月—2022年6月确诊为AAV的25例患儿的临床资料。 结果 25例患儿中，男性5例，女性20例；发病的中位年龄为11.0岁。泌尿系统受累18例（72%），呼吸系统受累10例（40%），皮肤受累6例（24%），眼、耳、鼻受累5例（20%），关节受累4例（16%），消化系统受累2例（8%）。11例完成肾脏穿刺，其中肾脏病理局灶型5例（46%），新月体型2例（18%），混合型2例（18%），硬化型2例（18%）；有免疫复合物沉积5例（45%）。7例达到慢性肾脏病（chronic kidney disease, CKD）Ⅴ期，其中2例死亡；2例完成肾移植。至随访期末，2例为CKD Ⅱ期，1例为CKD Ⅲ期。显微镜下多血管炎（microscopic polyangiitis, MPA）组16例，其中有13例（81%）累及泌尿系统；肉芽肿性多血管炎（granulomatosis with polyangiitis, GPA）组9例，其中6例（66%）有鼻窦炎；MPA组血清肌酐及尿酸较GPA组高（P<0.05），红细胞计数及肾小球滤过率较GPA组低（P<0.05）。 结论 儿童AAV好发于学龄期女性儿童，临床亚型以MPA多见。儿童AAV起病表现以肾脏受累为主，其次为呼吸系统受累。肾脏病理以局灶型多见，可伴有免疫复合物沉积。MPA患儿多有肾脏受累，GPA患儿鼻窦炎常见。AAV患儿预后不佳，多伴有肾功能不全。
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.
关键词
抗中性粒细胞胞质抗体相关性血管炎 /
临床表现 /
慢性肾脏病 /
预后 /
儿童
Key words
Anti-neutrophil cytoplasmic antibody-associated vasculitis /
Clinical manifestation /
Chronic kidney disease /
Prognosis /
Child
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参考文献
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基金
国家重点研发计划（ 2021YFC 2702004）。
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