摘要
目的研究儿童抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)合并肾损害的临床特点、病理变化和及其治疗效果。方法回顾性分析2018年11月至2023年3月海南省妇女儿童医学中心肾病风湿免疫科收治的7例AAV患儿的临床特点、病理检查结果和诊治资料。结果7例AAV患儿均有肺部受损(其中1例弥漫性肺泡出血,1例肺含铁血黄素沉积症,1例呈白肺样改变),6例有肾脏受累(其中1例进展为严重的硬化性肾小球肾炎,2例为不同程度肾小球和肾小管损害)。7例患儿中4例予糖皮质激素(GC)联合免疫抑制剂治疗,2例予GC联合免疫抑制剂、利妥昔单抗(RTX)治疗,1例予GC联合RTX治疗。最终6例有不同程度好转,1例因肾功能不全于家中突发严重高钾血症,心跳呼吸骤停死亡。结论AAV合并肾损害并非少见,临床医师应及早完善ANCA和肾活检明确诊断,早期予GC联合生物制剂治疗的效果优于GC联合免疫抑制剂治疗。
Objective To study the clinical features,pathological changes,and therapeutic effect of antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV)complicated with renal damage in children.Methods The clinical characteristics,pathological examination results,diagnosis,and treatment data of seven children with AAV admitted to the Department of Nephrology,Rheumatism and Immunology,Hainan Women and Children's Medical Center from November 2018 to March 2023 were retrospectively analyzed.Results Seven children with AAV had lung damage(including one case of diffuse alveolar hemorrhage,one case of pulmonary hemosiderosis,and one case of white lung like change),and six children had renal damage(including one case of severe sclerosing glomerulonephritis,and two cases of varying degrees of glomerular and tubular damage).Four cases were treated with glucocorticoid(GC)combined with immunosuppressive agents,two cases were treated with GC combined with immunosuppressive agents and rituximab(RTX),and one case was treated with GC combined with RTX.Finally,six cases were improved to varying degrees,and one case died of sudden severe hyperkalemia at home due to renal insufficiency and cardiac and respiratory arrest.Conclusion AAV complicated with renal damage is not rare.Clinicians should improve ANCA and renal biopsy as soon as possible to confirm the diagnosis.The effect of early GC combined with biological agents is better than GC combined with immunosuppressive therapy.
作者
陈金淑
徐志泉
宋依瑾
韦佳欣
孙婧
陈丕厚
CHEN Jin-shu;XU Zhi-quan;SONG Yi-jin;WEI Jia-xin;SUN Jing;CHEN Pi-hou(Department of Nephrology,Rheumatism and Immunology,Hainan Women's and Children's Medical Center,Haikou 570100,Hainan,CHINA)
出处
《海南医学》
CAS
2023年第19期2813-2818,共6页
Hainan Medical Journal
基金
海南省临床医学中心建设项目(编号:琼卫医函【2021】75号)
海南省优秀人才团队资助项目(编号:琼人才办通【2021】21号)。
关键词
儿童
抗中性粒细胞胞浆抗体相关性血管炎
肾损害
肺部病变
贫血
Children
Antineutrophil cytoplasmic antibody-associated vasculitis
Renal damage
Pulmonary lesions
Anemia
