Anti-p97/VCP antibodies: An autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease?
Anti-p97/VCP antibodies: An autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease?
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DOI：
10.1111/j.1365-3083.2006.01747.x
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发表时间：
2006-05-01
影响因子：
3.7
通讯作者：
Fritzler, MJ
中科院分区：
文献类型：
--
作者：
Miyachi, K;Hosaka, H;Fritzler, MJ
我们以前报道，12.5%的原发性胆汁性肝硬化（PBC）血清与95 kDa的胞质蛋白（p95 c），随后被确定为p97/valosin含蛋白（VCP）。对6例抗p97/VCP阳性PBC患者的临床特征和病程进行了平均15年的监测，这些患者均进行了Scheuer’s 1期和2期肝活检。将该组与50例未检测到抗VCP的PBC患者进行比较。用免疫沉淀法测定全长重组p97/VCP的自身抗体。所有6例PBC患者抗VCP抗体的线粒体丙酮酸脱氢酶复合物E2抗原测定的可寻址激光珠免疫。第一位是一位没有肝衰竭迹象的男性，在85岁时死于脑梗死。第二例是一名73岁的女性桥本甲状腺炎患者，在未接受熊去氧胆酸（UDCA）治疗的情况下临床保持稳定。虽然第三个没有HCV抗体，但他在76岁时患上了肝细胞癌，并在78岁时死于肾衰竭。第4例为50岁女性，随访期间临床稳定，第5例为桥本甲状腺炎，UCDA治疗后肝功能稳定。第六例为男性患者，临床病程轻微。这些患者的临床过程与对照组50例PBC患者谁没有抗p97/VCP相反。由于6例PBC患者抗p97/VCP抗体缓慢进展的肝脏疾病，没有死亡率相关的自身免疫性肝病，我们的观察表明，这种自身抗体可能是一个良好的预后指标。
We previously reported that 12.5% of primary biliary cirrhosis (PBC) sera reacted with a 95 kDa cytosol protein (p95c) that was subsequently identified as a p97/valosin-containing protein (VCP). The clinical features and course of the six anti-p97/VCP-positive PBC patients with Scheuer's stage 1 and 2 liver biopsies were monitored for an average of 15 years. This group was compared with 50 PBC patients that did not have detectable anti-VCP. Autoantibodies to a full-length recombinant p97/VCP were assayed by immunoprecipitation. All six PBC patients with anti-VCP had antibodies to the mitochondrial pyruvate dehydrogenase complex-E2 antigen as measured by an addressable laser bead immunoassay. The first was a male with no evidence of liver failure that died of cerebral infarction at the age of 85. The second was a 73-year-old female with Hashimoto's thyroiditis who has remained clinically stable without ursodeoxycolic acid (UDCA) treatment. Although the third had no HCV antibodies, he developed hepatocellular carcinoma at the age of 76 and died of renal failure at 78. The fourth was a 50-year-old female who remained clinically stable during follow-up and the fifth with Hashimoto's thyroiditis and stable liver function following UCDA treatment. The sixth was a male patient presenting a mild clinical course. The clinical course of these patients was in contrast to the 50 comparison group PBC patients who did not have anti-p97/VCP. As the six PBC patients with anti-p97/VCP antibodies had slowly progressive liver disease and no mortality related to autoimmune liver disease, our observations suggest that this autoantibody might be an indicator of a favourable prognosis.