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儿童 C-ANCA/蛋白酶 3 阳性结肠炎:一种具有独特表现的炎症性肠病还是以结肠炎为首发表现的血管炎?

C-ANCA/proteinase 3-positive colitis in children: a distinctive form of inflammatory bowel disease or vasculitis with colitis as initial presentation?

机构信息

*Paediatric Gastroenterology †Department of Clinical Chemistry, Microbiology and Immunology, Laboratory of Clinical Biology ‡Adult Gastroenterology, Ghent University Hospital, Belgium.

出版信息

J Pediatr Gastroenterol Nutr. 2013 Oct;57(4):489-92. doi: 10.1097/MPG.0b013e31829d4e9e.

DOI:10.1097/MPG.0b013e31829d4e9e
PMID:23752080
Abstract

AIM

Anti-neutrophil cytoplasmic antibodies (ANCAs) detected by indirect immunofluorescence have been found in patients with inflammatory bowel disease (IBD). Nevertheless, specific antibodies against proteinase-3 (PR3) are rare in this context.

METHODS

Sera from 30 consecutive pediatric patients with IBD were evaluated for ANCA-indirect immunofluorescence and its specific antibodies to investigate whether PR3-ANCA positivity (PR3-ANCA+) identifies a distinct IBD subtype.

RESULTS

The 5 PR3-ANCA+ patients (17%) showed significantly more concomitant biliary disease and severe anal blood loss (P < 0.05). None had vasculitis features at diagnosis nor during follow-up.

CONCLUSIONS

This pilot study demonstrates significant clinical differences between the PR3-ANCA-positive and -negative IBD subset.

摘要

目的

通过间接免疫荧光法检测到的抗中性粒细胞胞浆抗体(ANCA)已在炎症性肠病(IBD)患者中发现。然而,在这种情况下,针对蛋白酶 3(PR3)的特异性抗体很少见。

方法

评估了 30 例连续的儿科 IBD 患者的血清,以进行 ANCA-间接免疫荧光及其特异性抗体检测,以研究 PR3-ANCA 阳性(PR3-ANCA+)是否可确定一个独特的 IBD 亚型。

结果

5 例 PR3-ANCA+患者(17%)同时伴有明显更多的胆道疾病和严重的肛门血便(P < 0.05)。在诊断时和随访期间,均无血管炎特征。

结论

这项初步研究表明,PR3-ANCA 阳性和阴性的 IBD 亚组之间存在显著的临床差异。

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Diagnostics (Basel). 2023 Dec 16;13(24):3682. doi: 10.3390/diagnostics13243682.
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[Proteinase 3 (cANCA)-associated ulcerative colitis with possible inflammatory leukoencephalopathic CNS involvement].[蛋白酶3(cANCA)相关的溃疡性结肠炎伴可能的炎症性白质脑病中枢神经系统受累]
Nervenarzt. 2020 Aug;91(8):737-739. doi: 10.1007/s00115-020-00950-9.
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A Case of Cerebral Vasculitis Associated with Ulcerative Colitis.
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