A great mimicker of primary biliary cholangitis

A 59 year-old man without past medical history was referred with biochemical features of cholestasis (aspartate aminotransferase (AST) 117 U/L, alanine aminotransferase (ALT) 83 U/L, gamma-glutamyl transferase (GGT) 1307 U/L, alkaline phosphatase (AP) 1803 U/L, total bilirubin 0,7 mg/dL), a strongly positive antimitochondrial M2 antibody (AMA-M2) titer (88 U), weight loss and abdominal pain since several months. He did not take any medications, nor there was a history of alcohol abuse or sexual risk behavior. Upon presentation, clinical examination showed a rather cachectic patient (body mass index 19 kg/m²), without further abnormalities. As primary biliary cholangitis (PBC) was suspected, treatment with ursodeoxycholic acid had been initiated but did not improve the cholestasis nor the pain. Additional investigations didn’t reveal any other irregularities, except for a mildly dilated aortic sinus root of 42 mm. The histopathological findings of a liver biopsy are shown below (Figure 1). This showed granulomas with cholangitis and increased presence of neutrophils, which raised suspicion for an infectious cause.