An Interesting Case of Autoimmune Liver Disease

Abstract Autoimmune liver diseases (AILD) are part of a broad spectrum of liver diseases with autoimmune etiology, usually present individually but at times have overlapping features. We present the case of a 60-year-old lady presenting with fatigue, itching and right upper quadrant abdominal pain. Further investigation showed cholestatic pattern of liver enzymes and evidence of portal hypertension without any evidence of extrahepatic obstruction. Autoimmune markers and liver biopsy showed overlapping features of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC), which is a separate diagnosis known as overlap syndrome, but by applying appropriate criterion, we were able to make a definite diagnosis of PBC. Differentiating PBC from overlap syndrome was important as therapy of both are different.