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探索溃疡性结肠炎中抗中性粒细胞胞浆抗体(ANCA)的致病性:一例报告凸显进展为ANCA相关血管炎的风险

Exploring ANCA Pathogenicity in Ulcerative Colitis: A Case Report Highlighting the Risk of Progression to ANCA-Associated Vasculitis.

作者信息

Hamid Abir, Vandergheynst Frederic, Ilzkovitz Maxime

机构信息

Internal Medicine Department, Hôpital Erasme, Hôpital Universitaire de Bruxelles (HUB), Université Libre de Bruxelles (ULB), Brussels, Belgium.

出版信息

Eur J Case Rep Intern Med. 2025 May 5;12(5):005061. doi: 10.12890/2025_005061. eCollection 2025.

DOI:10.12890/2025_005061
PMID:40352707
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12061215/
Abstract

BACKGROUND

The relationship between anti-neutrophil cytoplasmic antibodies (ANCA) and inflammatory bowel disease has attracted significant attention due to shared immunopathological mechanisms and clinical associations.

CASE DESCRIPTION

This case report describes a 20-year-old African woman with a history of ulcerative colitis who developed diffuse alveolar haemorrhage in the setting of elevated proteinase 3 (PR3)-ANCA. She was treated for ANCA-associated vasculitis with corticosteroids, plasma exchange, and rituximab, leading to a complete resolution.

CONCLUSION

The case highlights the association between ulcerative colitis and ANCA-associated vasculitis, particularly with PR3-ANCA, and the importance of recognizing this overlap. Although ANCA are frequently present in inflammatory bowel disease, especially ulcerative colitis, their pathogenic role remains unclear. The incidence of ANCA-associated vasculitis is higher in inflammatory bowel disease patients, with ulcerative colitis often preceding ANCA-associated vasculitis. Despite the common presence of ANCA in inflammatory bowel disease, not all patients progress to ANCA-associated vasculitis. This case underscores the need for careful monitoring in ulcerative colitis patients with elevated PR3-ANCA and the role of ANCA in guiding diagnosis and treatment.

LEARNING POINTS

High proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibodies (ANCA) titre impacts the severity and management of ulcerative colitis patients.ANCA-associated vasculitis is more frequent in ulcerative colitis patients and occurs several years after ANCA positivity.Close monitoring of ulcerative colitis patients with PR3-ANCA is essential for early detection of progression to ANCA-associated vasculitis.

摘要

背景

由于存在共同的免疫病理机制和临床关联,抗中性粒细胞胞浆抗体(ANCA)与炎症性肠病之间的关系已引起广泛关注。

病例描述

本病例报告描述了一名20岁有溃疡性结肠炎病史的非洲女性,在蛋白酶3(PR3)-ANCA升高的情况下发生了弥漫性肺泡出血。她接受了糖皮质激素、血浆置换和利妥昔单抗治疗ANCA相关性血管炎,最终完全缓解。

结论

该病例突出了溃疡性结肠炎与ANCA相关性血管炎之间的关联,尤其是与PR3-ANCA的关联,以及认识到这种重叠的重要性。虽然ANCA在炎症性肠病尤其是溃疡性结肠炎中经常出现,但其致病作用仍不明确。炎症性肠病患者中ANCA相关性血管炎的发病率较高,溃疡性结肠炎常先于ANCA相关性血管炎出现。尽管ANCA在炎症性肠病中普遍存在,但并非所有患者都会发展为ANCA相关性血管炎。该病例强调了对PR3-ANCA升高的溃疡性结肠炎患者进行仔细监测的必要性以及ANCA在指导诊断和治疗中的作用。

学习要点

高蛋白酶3(PR3)-抗中性粒细胞胞浆抗体(ANCA)滴度影响溃疡性结肠炎患者的严重程度和治疗。ANCA相关性血管炎在溃疡性结肠炎患者中更常见,且在ANCA阳性数年后发生。对PR3-ANCA阳性的溃疡性结肠炎患者进行密切监测对于早期发现进展为ANCA相关性血管炎至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8495/12061215/3e47b0e5c6a9/5061_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8495/12061215/3e47b0e5c6a9/5061_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8495/12061215/3e47b0e5c6a9/5061_Fig1.jpg

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Semin Arthritis Rheum. 2024 Jun;66:152452. doi: 10.1016/j.semarthrit.2024.152452. Epub 2024 Apr 23.
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Role of Serum Proteinase 3 Antineutrophil Cytoplasmic Antibodies in the Diagnosis, Evaluation of Disease Severity, and Clinical Course of Ulcerative Colitis.
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Serum ANCA as Disease Biomarkers: Clinical Implications Beyond Vasculitis.血清抗中性粒细胞胞浆抗体作为疾病生物标志物:血管炎之外的临床意义。
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Anti-proteinase 3 antineutrophil cytoplasmic antibody reflects disease activity and predicts the response to steroid therapy in ulcerative colitis.抗蛋白酶 3 抗中性粒细胞胞质抗体反映疾病活动度,并预测溃疡性结肠炎对类固醇治疗的反应。
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