Primary Biliary Cholangitis- Autoimmune Hepatitis Overlap Syndrome

Excerpt

To the Editor: The prevalence of Primary biliary cholangitis- autoimmune hepatitis (PBC-AIH) overlap syndrome is approximately 2% in adults initially diagnosed as AIH [1]. We present a case of 16-y-old girl who presented with history of itching and darkening of skin for 4 y, without jaundice, abdominal pain, fever or hepatotoxic drug ingestion. She then developed jaundice over the next one month without hepatic de-compensation. Patient had elevated direct bilirubin, alkaline phosphatase, alanine aminotransferase and normal prothrombin time. Her viral markers, autoimmune antibodies, and ceruloplasmin were negative. Her anti mitochondrial antibody (AMA-M2, 1:100 titer) was positive. Her serum immunoglobulin G (IgG- 2200 mg/dl) and IgM (368 mg/dl) levels were elevated. Her Magnetic resonance cholangiopancreatography (MRCP) was normal. Serial Liver function tests (LFT) are depicted in Table 1 below. …