Florid biliary duct lesions in an AMA -positive patient in absence of cholestatic liver biochemistry.

J Autoimmun

Gastroenterology & Hepatology Department Hamad Medical Corporation, Qatar; Weill Cornell Medical College, Qatar Br, Qatar. Electronic address:

Published: July 2019

AI Article Synopsis

  • Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease diagnosed through specific antibodies and liver function tests.
  • The case presented features granulomatous lymphocytic cholangitis with positive AMA, Anti-M2, and anti-nuclear antibodies, despite normal levels of alkaline phosphatase (ALP).
  • Contrary to past studies, the patient’s persistently normal ALP did not indicate a benign course, as they ultimately progressed to liver cirrhosis.

Article Abstract

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease, diagnosed by the presence of anti-mitochondrial antibodies (AMA) or highly PBC-specific anti-nuclear antibodies, in the appropriate context of cholestatic liver biochemistry. We present a case with histological features of destructive granulomatous lymphocytic cholangitis affecting interlobular and septal bile ducts suggestive of PBC, with strong positive AMA, Anti-M2 and anti-nuclear dot, but with persistently normal alkaline phosphatase (ALP). On the contrary to previous reports suggesting that those individuals in whom ALP remains persistently below 1.5 times ULN appear to have a benign course and a better prognosis, our patient progressed to liver cirrhosis.

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http://dx.doi.org/10.1016/j.jaut.2019.04.004DOI Listing

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