1型自身免疫性肝炎中的高滴度抗中性粒细胞胞浆抗体

High-titer antineutrophil cytoplasmic antibodies in type-1 autoimmune hepatitis.

作者信息

Targan S R, Landers C, Vidrich A, Czaja A J

机构信息

Cedars-Sinai Inflammatory Bowel Disease Center, Cedars-Sinai Medical Center, Los Angeles, California.

出版信息

Gastroenterology. 1995 Apr;108(4):1159-66. doi: 10.1016/0016-5085(95)90215-5.

DOI:10.1016/0016-5085(95)90215-5

PMID:7698584

Abstract

BACKGROUND/AIMS: Autoimmune hepatitis (AIH), an unresolving liver inflammation characterized by periportal hepatitis and presence of serum autoantibodies, is distinguished by smooth muscle antibody and/or antinuclear antibody seropositivity. Type-2 AIH is characterized by antibodies to liver/kidney microsome type-1. Antineutrophil cytoplasmic antibodies (ANCAs) are highly specific for ulcerative colitis, primary sclerosing cholangitis, and, in this report, type-1 AIH determined by positive enzyme-linked immunosorbent assay confirmed by perinuclear indirect immunofluorescence staining. The studies described characterize the frequency and nature of ANCA in severe type-1 AIH patients and define AIH-ANCA specificity and diagnostic significance.

METHODS

One hundred four patients, characterized by clinical, immunoserological, virological, histological, and antigenic criteria, were studied. ANCA expression was assayed by enzyme-linked immunosorbent assay.

RESULTS

High-titer ANCA is present in 96% of patients with type-1 AIH with 92% showing a perinuclear staining pattern (pANCA). Whereas many patients were seropositive for antinuclear antibodies, the titer of ANCA did not correlate with the titer of antinuclear antibodies. ANCA expression did not correlate with the presence or absence of other autoimmune disorders. Finally, 80% of patients with AIH examined expressed only immunoglobulin G1 pANCA contrasting with the 33% of patients with PSC with pANCA subclass specificity.

CONCLUSIONS

The presence of ANCA seems to be an independent and selective marker for type-1 AIH.

摘要

背景/目的：自身免疫性肝炎（AIH）是一种以门周肝炎和血清自身抗体存在为特征的持续性肝脏炎症，以平滑肌抗体和/或抗核抗体血清阳性为特点。2型AIH以抗肝肾微粒体1型抗体为特征。抗中性粒细胞胞浆抗体（ANCA）对溃疡性结肠炎、原发性硬化性胆管炎具有高度特异性，在本报告中，通过酶联免疫吸附测定呈阳性并经核周间接免疫荧光染色确认的1型AIH也具有该抗体。所述研究描述了严重1型AIH患者中ANCA的频率和性质，并确定了AIH-ANCA的特异性和诊断意义。

方法

对104例根据临床、免疫血清学、病毒学、组织学和抗原标准确定的患者进行研究。通过酶联免疫吸附测定法检测ANCA表达。

结果

96%的1型AIH患者存在高滴度ANCA，其中92%显示核周染色模式（pANCA）。虽然许多患者抗核抗体血清呈阳性，但ANCA滴度与抗核抗体滴度无关。ANCA表达与其他自身免疫性疾病的有无无关。最后，80%接受检查的AIH患者仅表达免疫球蛋白G1 pANCA，而原发性硬化性胆管炎患者中具有pANCA亚类特异性的患者比例为33%。