Department of Paediatrics, University Hospital and Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Institute of Immunology, Technische Universität Dresden, Dresden, Germany.
J Pediatr Gastroenterol Nutr. 2022 Apr 1;74(4):463-470. doi: 10.1097/MPG.0000000000003359. Epub 2021 Nov 24.
Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis, but is also found in patients with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). The aim of our study was to investigate ANCA and PR3-ANCA in paediatric IBD.
We tested 326 paediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence, IIF) and PR3-ANCA (chemiluminescence immunoassay). We applied the Paris classification for paediatric IBD and documented liver manifestations such as primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH).
We found PR3-ANCA in 49/121 (40%) of UC, 20/187 (11%) of Crohn disease (CD) and 2/18 (11%) of IBD-unclassified (IBD-U) patients but in none of the controls. 54% UC and 12% CD patients were positive for ANCA (IIF). PR3-ANCA positive UC patients were characterised by more extensive disease (P = .070). Fourteen of 21 (67%) of UC patients with backwash ileitis were anti-PR3 ANCA-positive (P = .011). We diagnosed PSC or PSC/AIH in 19 UC and 3 IBD-U patients. Fifteen of 22 (68%) patients with PSC or PSC/AIH were anti-PR3-ANCA positive in contrast to 36 of 117 (32%) patients without PSC (P = .001). PR3-ANCA positive patients showed higher levels of gamma-glutamyl transferase, alanine transaminase and aspartate transferase (P < 0.001, 0.001, 0.006, respectively). Forty-seven percent of CD and 6% of UC patients were ASCA-IgA positive. PR3-ANCA-positive and -negative patients showed no significant differences concerning age at diagnosis, disease activity, need for drugs, and number of hospitalisations.
Our study provides data for PR3-ANCA as a potential serological marker for paediatric UC and PSC.
抗中性粒细胞胞浆抗体（ANCA）针对蛋白酶 3（PR3）是肉芽肿性多血管炎的标志物，但也存在于炎症性肠病（IBD）患者中，主要是溃疡性结肠炎（UC）。我们研究的目的是研究儿科 IBD 中的 ANCA 和 PR3-ANCA。
我们检测了 326 名儿科 IBD 患者和 164 名对照者的抗酿酒酵母抗体（ASCA）、ANCA（间接免疫荧光，IIF）和 PR3-ANCA（化学发光免疫分析）。我们应用了儿科 IBD 的巴黎分类，并记录了原发性硬化性胆管炎（PSC）和自身免疫性肝炎（AIH）等肝脏表现。
我们发现 49/121（40%）UC、20/187（11%）克罗恩病（CD）和 2/18（11%）IBD 未分类（IBD-U）患者的 PR3-ANCA 阳性，但对照组均为阴性。54%UC 和 12%CD 患者的 ANCA（IIF）阳性。PR3-ANCA 阳性 UC 患者的疾病更广泛（P=0.070）。21 例回肠反流性结肠炎 UC 患者中有 14 例（67%）抗 PR3 阳性（P=0.011）。我们诊断 19 例 UC 和 3 例 IBD-U 患者为 PSC 或 PSC/AIH。PSC 或 PSC/AIH 患者中有 15 例（68%）抗 PR3-ANCA 阳性，而无 PSC 的 117 例患者中仅有 36 例（32%）（P=0.001）。PR3-ANCA 阳性患者的γ-谷氨酰转移酶、丙氨酸转氨酶和天冬氨酸转氨酶水平更高（P<0.001、0.001、0.006）。47%CD 和 6%UC 患者的 ASCA-IgA 阳性。PR3-ANCA 阳性和阴性患者在诊断时的年龄、疾病活动度、药物需求和住院次数方面无显著差异。
我们的研究为 PR3-ANCA 作为儿科 UC 和 PSC 的潜在血清学标志物提供了数据。
