Clinical and pathological features of primary biliary cholangitis with negative anti-mitochondrial antibody
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摘要:
抗线粒体抗体(AMA)是原发性胆汁性胆管炎(PBC)的典型血清标志物,临床易忽视AMA阴性PBC的诊断。分析了AMA阴性与阳性PBC的流行病学、临床及病理特征、治疗效果差异,认为二者在瘙痒症状、IgM、胆管损伤水平差异较明显。临床诊断AMA阴性PBC患者,需通过联合免疫荧光法及免疫学检测以排除AMA假阴性,结合胆汁淤积表现、抗sp100、gp210、ANA抗体阳性及病理检查的典型旺炽性胆管炎等改变明确诊断,并与其他胆管损伤或缺失疾病鉴别。
Abstract:Anti-mitochondrial antibody ( AMA) is a typical serum marker for primary biliary cholangitis ( PBC) , and the diagnosis ofAMA-negative PBC may easily be neglected in clinical practice. This article analyzes the differences in epidemiology, clinical and patho-logical features, and treatment outcome between AMA-negative and AMA-positive PBC and points out that there are significant differencesbetween them in the symptom of pruritus, immunoglobulin M, and severity of bile duct injury. For patients with a clinical diagnosis of AMA-negative PBC, immunofluorescence assay combined with immunological detection should be performed to exclude the false-negative resultof AMA, and a confirmed diagnosis can be made with reference to the manifestation of cholestasis, positive results of anti-sp100, anti-gp210, and anti-ANA antibodies, and typical hyperactive cholangitis based on pathological examination. Differential diagnosis of this dis-ease with other types of bile duct injury or absence of bile duct should be taken seriously in clinical practice.
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