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自身免疫性肝炎中胆管损伤的诊断和治疗意义

Diagnostic and therapeutic implications of bile duct injury in autoimmune hepatitis.

作者信息

Czaja Albert J, Muratori Paolo, Muratori Luigi, Carpenter Herschel A, Bianchi Francesco B

机构信息

Division of Gastroenterology and Hepatology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.

出版信息

Liver Int. 2004 Aug;24(4):322-9. doi: 10.1111/j.1478-3231.2004.0924.x.

Abstract

BACKGROUND

Bile duct injury is not a feature of classical autoimmune hepatitis (AIH), but it has been described in variant forms of the disease.

AIMS

Our goals were to assess the similarity of AIH with bile duct injury to classical disease and to evaluate the possibility of concurrent primary biliary cirrhosis (PBC).

METHODS

Fifteen patients with bile duct injury were compared with 151 patients with classical AIH. Patterns of nuclear immunofluorescence and the frequency and nature of autoantibodies associated with AIH and PBC were determined.

RESULTS

Patients with bile duct injury had the same nuclear-staining patterns, frequency and nature of autoantibodies, and genetic risk factors as the comparison group. Features specific for PBC, including the multiple nuclear dot pattern of immunofluorescence and antibodies to the M2 antigens, Sp100 and nuclear pore complex antigen, gp210, did not distinguish them from classical disease. Remission and treatment failure occurred with similar frequencies in both groups.

CONCLUSIONS

Patients with AIH and bile duct injury lack features of PBC, and they respond as well to corticosteroid therapy as patients with classical disease. Background bile duct changes should not alter the diagnosis or treatment of AIH.

摘要

背景

胆管损伤并非经典自身免疫性肝炎(AIH)的特征,但在该病的变异形式中已有描述。

目的

我们的目标是评估伴有胆管损伤的AIH与经典疾病的相似性,并评估并发原发性胆汁性肝硬化(PBC)的可能性。

方法

将15例胆管损伤患者与151例经典AIH患者进行比较。确定核免疫荧光模式以及与AIH和PBC相关的自身抗体的频率和性质。

结果

胆管损伤患者与对照组具有相同的核染色模式、自身抗体频率和性质以及遗传风险因素。PBC的特异性特征,包括免疫荧光的多核点模式以及针对M2抗原、Sp100和核孔复合体抗原gp210的抗体,并未将他们与经典疾病区分开来。两组缓解和治疗失败的发生率相似。

结论

伴有胆管损伤的AIH患者缺乏PBC的特征,并且他们对皮质类固醇治疗的反应与经典疾病患者一样好。背景胆管改变不应改变AIH的诊断或治疗。

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