TY - JOUR
T1 - Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase
T2 - Is alkaline phosphatase truly a surrogate marker?
AU - Sun, Chunyan
AU - Xiao, Xiao
AU - Yan, Li
AU - Sheng, Li
AU - Wang, Qixia
AU - Jiang, Pan
AU - Lian, Min
AU - Li, Yanmei
AU - Wei, Yiran
AU - Zhang, Jun
AU - Chen, Yong
AU - Li, Bo
AU - Li, You
AU - Huang, Binyuan
AU - Li, Yikang
AU - Peng, Yanshen
AU - Chen, Xiaoyu
AU - Fang, Jingyuan
AU - Qiu, Dekai
AU - Hua, Jing
AU - Tang, Ruqi
AU - Leung, Patrick S
AU - Gershwin, M. Eric
AU - Miao, Qi
AU - Ma, Xiong
PY - 2019/5/1
Y1 - 2019/5/1
N2 - 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.
AB - 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.
KW - Alkaline phosphatase
KW - Antimitochondrial antibody
KW - Cholangitis activity
KW - Histology
KW - Primary biliary cholangitis
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U2 - 10.1016/j.jaut.2019.01.005
DO - 10.1016/j.jaut.2019.01.005
M3 - Article
C2 - 30709684
AN - SCOPUS:85064200389
VL - 99
SP - 33
EP - 38
JO - Journal of Autoimmunity
JF - Journal of Autoimmunity
SN - 0896-8411
ER -