CAJ | 학술논문

目的探讨儿童抗中性粒细胞胞浆抗体相关性系统性血管炎的临床、病理特点及预后.方法回顾性分析15例儿童抗中性粒细胞胞浆抗体相关性系统性血管炎的临床资料.结果女11例,男4例,平均年龄10.7岁,14例为显微镜下型多血管炎.确诊时间0.5 ～40个月.15铡患儿均有血尿和蛋白尿,13例诊断时肾功能下降,其中8例达终末肾.12例曾行肾活检,11例(92％)肾脏病理可见新月体形成,以细胞纤维或纤维性新月体为主,其中6例(50％)诊断为新月体性肾炎,仅2例临床表现为急进性肾炎.10例肾组织可见不同程度硬化,3例诊断为硬化性肾小球肾炎.肾外表现主要为贫血和呼吸系统受累.14例患儿予激素联合免疫抑制剂治疗,3例联合血浆置换.14例短期随访,诊断时肌酐清除率＜30 ml/(min·1.73 m2)者10例,肾脏病理类型为新月体肾炎或硬化性肾小球肾炎,肾功能均未恢复.结论对于不明原因贫血和肺部病变的学龄期女孩,建议早期检查ANCA和肾功能,如有异常早期行肾活检.抗中性粒细胞胞浆抗体相关性系统性血管炎患儿肾脏损害严重,临床隐匿,肌酐清除率＜30 ml/(min·1.73 m2)者预后差.
목적 탐토인동항중성립세포포장항체상관성계통성혈관염적림상、병리특점급예후.방법 회고성분석15례인동항중성립세포포장항체상관성계통성혈관염적림상자료.결과 녀11례,남4례,평균년령10.7세,14례위현미경하형다혈관염.학진시간0.5 ～40개월.15찰환인균유혈뇨화단백뇨,13례진단시신공능하강,기중8례체종말신.12례증행신활검,11례(92％)신장병리가견신월체형성,이세포섬유혹섬유성신월체위주,기중6례(50％)진단위신월체성신염,부2례림상표현위급진성신염.10례신조직가견불동정도경화,3례진단위경화성신소구신염.신외표현주요위빈혈화호흡계통수루.14례환인여격소연합면역억제제치료,3례연합혈장치환.14례단기수방,진단시기항청제솔＜30 ml/(min·1.73 m2)자10례,신장병리류형위신월체신염혹경화성신소구신염,신공능균미회복.결론 대우불명원인빈혈화폐부병변적학령기녀해,건의조기검사ANCA화신공능,여유이상조기행신활검.항중성립세포포장항체상관성계통성혈관염환인신장손해엄중,림상은닉,기항청제솔＜30 ml/(min·1.73 m2)자예후차.
Objective Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis.This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children,to analyze the clinical features,pathological characteristics and the prognosis of children with ANCA-associated vasculitis.Method Fifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included.Their data of pre-diagnosis status,clinical manifestations,renal pathology,treatment and prognosis were reviewed retrospectively.Result Of the 15 children,11 were girls and 4 boys with a mean age of 10.7 years.Fourteen children were categorized as microscopic polyangitis.The time to diagnosis varied from 0.5 month to 40 months.Hematuria and proteinuria were revealed by urine analysis in all of them,only 6 children complained with gross hematuria or edema of oliguria.Decreased glomerular filtration rate was revealed in 13 children,8 of whom had a creatinine clearance rate of less than 15 ml/(min · 1.73 m2).Twelve children underwent renal biopsy,crescent formation was found in 11 children.Most of the crescents were cellular fibrous crescents or fibrous crescents.Six children were diagnosed as crescentic nephritis; the process of rapidly progressive nephritis was only observed in 2 children.Segmental glomerulosclerosis or global glomerulosclerosis were found in 10 children,3 of them were diagnosed as sclerotic glomerulonephritis.Anemia and pulmonary injury were the most common extra renal manifestations.Other extra renal manifestations included rash,pain joint,gastrointestinal symptoms,abnormal findings of cardiac ultrasonography and headache.Eight children were treated with steroid combined with cyclophosphamide,4 were treated with steroid and mycophenolate mofetil,2 were treated with steroid,cyclophosphamide and mycophenolate mofetil,3 children were treated with plasma exchange.Fourteen children were followed up for 0.5 month to 4 years.The renal function did not recover in children with crcatinine clearance rate of less than 30 ml/(min · 1.73 m2),who showed crescentic glomerulonephritis or sclerotic glomerulonephritis.The children who had creatinine clearance rate of more than 30 ml/(min · 1.73 m2) had better prognosis.Conclusion More attention should be paid to ANCA-associated vasculitis among school age girls with anemia or pulmonary diseases.The renal damage was serious in children; however,the clinical manifestations were not obvious.Children with a creatinine clearance rate of less than 30 ml/(min · 1.73 m2) had poor prognosis.Early accurate diagnosis is very important.