Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase: Is alkaline phosphatase truly a surrogate marker?

Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase: Is alkaline phosphatase truly a surrogate marker?
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组织学证明 AMA 阳性原发性胆汁性胆管炎,但血清碱性磷酸酶正常:碱性磷酸酶真的是替代标志物吗?

DOI:
10.1016/j.jaut.2019.01.005
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发表时间:
2019-05-01
影响因子:
12.8
通讯作者:
Ma, Xiong
Ma, Xiong
中科院分区:
医学1区
文献类型:
--
作者:
Sun, Chunyan;Xiao, Xiao;Ma, Xiong

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背景和目标:抗线粒体抗体(AMAs)是原发性胆管炎(PBC)的血清学标志,是人类免疫病理学中最具针对性和特异性的自身抗体。然而,发现AMA阳性且碱性磷酸酶(ALP)正常的临床意义仍然是个谜。方法:我们利用了2012年1月至2018年1月期间被确定为AMA阳性但ALP水平正常的169名连续门诊患者。67/169这些AMA阳性正常ALP patients.Results:在所有169例患者中,我们再次确认AMA,并进行抗gp 210和抗sp100,肝脏硬度(LSM)评估振动控制瞬态弹性成像(VCTE),腹部计算机断层扫描(CT)扫描,无论是磁共振成像(MRI)或超声。由两名公正的观察员审查肝活检。169例患者中87.6%为女性,平均年龄46岁,中位AMA滴度为1:320,血清IgM升高者占53.3%。重要的是,在肝活检患者中,55(82.1%)的67有不同程度的胆管炎活动,诊断PBC.Conclusion:在AMA阳性但ALP水平正常的患者中,超过80%与组织学经典PBC相关。这些数据强调了AMA阳性的重要性,即使ALP正常,也质疑ALP作为胆管炎唯一替代标志物的作用。
Background and aims: The most highly directed and specific autoantibody in human immunopathology is the serologic hallmark of primary biliary cholangitis (PBC), antimitochondrial antibodies (AMAs). However the clinical significance of finding a positive AMA, with normal alkaline phosphatase (ALP) remains enigmatic.Methods: We took advantage of 169 consecutive outpatients who were identified as having a positive AMA, but normal ALP levels between January 2012 and January 2018. A liver biopsy was performed on 67/169 of these AMA positive normal ALP patients.Results: In all 169 patients we reconfirmed the AMA and also performed anti-gp210 and anti-sp100, liver stiffness (LSM) assessed by vibration-controlled transient elastography (VCTE), an abdominal computed tomography (CT) scan, and either a magnetic resonance imaging (MRI) or ultrasound. The liver biopsies were reviewed by two unbiased observers. 87.6% of the 169 patients were females with a mean age of 46; the median AMA titer 1:320; an elevated serum IgM was found in 53.3%. Importantly, in patients with a liver biopsy, 55 ( 82.1%) out of 67 had varying degrees of cholangitis activity, diagnostic of PBC.Conclusion: In patients who were AMA-positive but had normal ALP levels, more than 80% were associated with histological classic PBC. These data emphasize the importance of a positive AMA, even with a normal ALP and also question the role of ALP as a sole surrogate marker of cholangitis.