Simranpreet K Mann, Jeffrey N Bone, Else S Bosman, David A Cabral, Kimberly A Morishita, Kelly L Brown
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By 1 year, there were no significant differences, based on ANCA positivity or specificity, in the likelihood of achieving inactive disease (~68%), experiencing improvement (≥87%) or acquiring damage (~58%). Similarly, and in contrast to adult-onset ANCA-associated vasculitis, there were no significant differences in the likelihood of having a relapse (~11%) between 1 and 2 years after diagnosis. Relative to PR3-ANCA, MPO-ANCA seropositivity was associated with a higher likelihood of kidney involvement (OR 2.4, 95% CI 1.3 to 4.7, p=0.008) and severe kidney dysfunction (Kidney Disease Improving Global Outcomes (KDIGO) stages 4-5; OR 6.04, 95% CI 2.77 to 13.57, p<0.001) at onset. 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引用次数: 0
摘要
研究目的本研究的目的是评估诊断时抗中性粒细胞胞浆抗体(ANCA)血清阳性和抗原特异性是否对儿科发病的小血管炎具有预测作用:根据髓过氧化物酶(MPO)(129人)和蛋白酶-3(PR3)ANCA的缺失(41人)或存在(236人)对患有小血管炎的儿童和青少年(406人)进行分层,比较诊断时的总体疾病活动性和肾脏特异性疾病活动性以及1至2年的预后,使用ARChiVe/儿科血管炎倡议登记处的回顾性临床数据拟合广义线性模型:结果:与ANCA阴性的血管炎患者相比,PR3-ANCA和MPO-ANCA血清阳性患者在确诊时的总体疾病活动度更高。到 1 年时,根据 ANCA 阳性或特异性,实现非活动性疾病(约 68%)、病情改善(≥ 87%)或获得损害(约 58%)的可能性没有显著差异。同样,与成人发病型 ANCA 相关性血管炎相比,确诊后 1 至 2 年间复发的可能性(约 11%)也没有显著差异。相对于 PR3-ANCA,MPO-ANCA 血清阳性与肾脏受累(OR 2.4,95% CI 1.3 至 4.7,p=0.008)和严重肾功能不全(肾病改善全球结局(KDIGO)4-5 期;OR 6.04,95% CI 2.77 至 13.57,p=0.008)的可能性较高相关:本研究结果表明,与成人患者相比,儿科患者的ANCA预测价值存在重大差异,因此在对这一人群做出治疗决定时应加以考虑。
Predictive utility of ANCA positivity and antigen specificity in the assessment of kidney disease in paediatric-onset small vessel vasculitis.
Objectives: The objective of this study is to evaluate whether anti-neutrophil cytoplasmic antibody (ANCA) seropositivity and antigen specificity at diagnosis have predictive utility in paediatric-onset small vessel vasculitis.
Methods: Children and adolescents with small vessel vasculitis (n=406) stratified according to the absence (n=41) or presence of ANCA for myeloperoxidase (MPO) (n=129) and proteinase-3 (PR3) (n=236) were compared for overall and kidney-specific disease activity at diagnosis and outcomes between 1 and 2 years using retrospective clinical data from the ARChiVe/Paediatric Vasculitis Initiative registry to fit generalised linear models.
Results: Overall disease activity at diagnosis was higher in PR3-ANCA and MPO-ANCA-seropositive individuals compared with ANCA-negative vasculitis. By 1 year, there were no significant differences, based on ANCA positivity or specificity, in the likelihood of achieving inactive disease (~68%), experiencing improvement (≥87%) or acquiring damage (~58%). Similarly, and in contrast to adult-onset ANCA-associated vasculitis, there were no significant differences in the likelihood of having a relapse (~11%) between 1 and 2 years after diagnosis. Relative to PR3-ANCA, MPO-ANCA seropositivity was associated with a higher likelihood of kidney involvement (OR 2.4, 95% CI 1.3 to 4.7, p=0.008) and severe kidney dysfunction (Kidney Disease Improving Global Outcomes (KDIGO) stages 4-5; OR 6.04, 95% CI 2.77 to 13.57, p<0.001) at onset. Nonetheless, MPO-ANCA seropositive individuals were more likely to demonstrate improvement in kidney function (improved KDIGO category) within 1 year of diagnosis than PR3-ANCA seropositive individuals with similarly severe kidney disease at onset (p<0.001).
Conclusions: The results of this study suggest important paediatric-specific differences in the predictive value of ANCA compared with adult patients that should be considered when making treatment decisions in this population.
期刊介绍:
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