Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis
Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis
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DOI：
10.1002/hep.510250507
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发表时间：
1997-05-01
期刊：
影响因子：
13.5
通讯作者：
Podda, M
中科院分区：
文献类型：
--
作者：
Invernizzi, P;Crosignani, A;Podda, M
来自北美和北欧的报道表明，抗线粒体抗体(AMA)阴性的原发性胆汁性肝硬变(PBC)是一种独特的慢性淤胆性肝病，除AMA外，血清非器官特异性自身抗体的发生率很高。为了评估这种特殊的血清免疫反应是否与临床相关特征有关，我们回顾了从1974年6月至1994年6月在我中心进行临床和组织学诊断并定期随访的297例意大利患者的经验。对AMA阴性和AMA阳性患者的生化和临床特征以及疾病的临床转归进行了比较。在297名患者中，30名(10%)的AMA间接免疫荧光检测为阴性。免疫印迹法检测其中6例抗线粒体M2抗体(AMA-M2)阳性，24例(8%)诊断为AMA阴性PBC。初诊时，AMA阴性和AMA阳性患者的生化和临床特征相似，抗核和抗平滑肌抗体(ANA和ASMA)在AMA阴性患者中阳性率更高(分别为718%和31%，37%和9%；均P=.0002)，并发症发生率和肝功能衰竭导致死亡或转诊为肝移植的两组无显著差异。总而言之，这项历史队列研究的数据表明，AMA阴性PBC的独特血清学特征与临床谱系或病程的显著差异无关。
Reports from North America and Northern Europe have suggested that antimitochondrial antibody (AMA) negative primary biliary cirrhosis (PBC) is a distinct chronic cholestatic liver disease with high prevalence of serum non-organ-specific autoantibodies other than AMA, To evaluate if such a peculiar serum immunoreactivity is associated with clinically relevant characteristics, we reviewed our experience with 297 Italian patients who have had a clinical and histological diagnosis of PBC and were regularly followed up at our Center from June 1974 to June 1994. AMA-negative and AMA-positive patients were compared in terms of biochemical and clinical features, and clinical outcome of the disease, At presentation, 30 of 297 patients (10%) tested negative for AMA by indirect immunofluorescence. Six of them tested positive for antimitochondrial M2 antibodies (AMA-M2) by immunoblotting analysis, therefore, diagnosis of AMA-negative PBC was made in 24 patients (8%), At the initial visit, AMA-negative and AMA-positive patients were similar in terms of biochemical and clinical features, Antinuclear and anti-smooth-muscle antibodies (ANA and ASMA) were more frequently positive in the AMA-negative patients (718 vs. 31%, and 37% vs. 9%; both P = .0002), Incidence of complications of cirrhosis and development of liver failure resulting in death or referral for liver transplantation did not differ significantly between the two populations. In conclusion, data from this historical cohort study suggest that the distinct serological features of AMA-negative PBC are not associated with substantial differences in the clinical spectrum or course of the disease.