Primary biliary cirrhosis (PBC) is an autoimmune cholestatic disease of the liver which affects mainly middle-aged women characterized by progressive destruction and loss of the small intrahepatic bile ducts which in turn, may lead to end-stage liver disease. The typical clinical phenotype is characterized by a middle-aged female with elevated cholestatic enzymes and positive antimitochondrial antibodies (AMA). However, apart from this typical presentation, there are important variants in everyday clinical practice. These variants include the AMA-negative PBC, the isolated AMA positivity, the AMA-positivity in patients with well-established autoimmune hepatitis (AIH), the premature ductopenic PBC variant and the PBC variant with characteristics of AIH (PBC-AIH variant). In this mini-review, we summarize and discuss the literature data and our own experience on the PBC variants highlighting also the uncertainties and a potential new era of the research agenda
