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

• Published in: Journal of autoimmunity 2019-07, Vol.101, p.153-155
• Main Authors: Sultan, Khaleel, Petkar, Mahir, Derbala, Moutaz
• Format: Article
• Language: English
• ISSN: 0896-8411; 1095-9157
• DOI: 10.1016/j.jaut.2019.04.004

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. •Patients with positive AMA and normal cholestatic liver enzymes, namely ALP, should still undergo stringent evaluation for PBC.•This may even include performing a liver biopsy, although, this should mainly be considered in patients with high titer of AMA and significantly elevated IgM level.•More studies are needed to clarify how to best monitor these patients as the traditional marker currently used to monitor disease progression is normal.•Different markers indicating disease progression need to be established and these can be then used to monitor response to therapy instead of ALP.•In addition, we do not know for certain whether these patients should be treated similarly to classic PBC or with a different strategy of treatment.•For example combination therapy with the ursodeoxycholic and Obeticholic acid is a better strategy to prevent progression of disease.•Further follow, up can shed some light on the differences between these patients as compared to patients with the classic form of PBC.