ANCA 阳性和抗原特异性在评估儿科小血管炎肾脏疾病中的预测作用。

IF 5.1 2区 医学 Q1 RHEUMATOLOGY
Simranpreet K Mann, Jeffrey N Bone, Else S Bosman, David A Cabral, Kimberly A Morishita, Kelly L Brown
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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。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.

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来源期刊
RMD Open
RMD Open RHEUMATOLOGY-
CiteScore
7.30
自引率
6.50%
发文量
205
审稿时长
14 weeks
期刊介绍: RMD Open publishes high quality peer-reviewed original research covering the full spectrum of musculoskeletal disorders, rheumatism and connective tissue diseases, including osteoporosis, spine and rehabilitation. Clinical and epidemiological research, basic and translational medicine, interesting clinical cases, and smaller studies that add to the literature are all considered.
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