Primary biliary cholangitis
Primary biliary cholangitis (PBC) is another autoimmune liver disease which targets the very small bile ducts. Despite the similarities to PSC, it’s not the same disease and is not to be confused with PSC. PBC and PSC are very different conditions.
Whereas PSC affects both the small and medium/large bile ducts, PBC only affects the small bile ducts within the liver itself and is not associated with scarring in the bile ducts 50.
Unlike PSC, PBC does not share an association with IBD, bowel cancer or bile duct cancer. However, individuals with PBC can occasionally develop thyroid problems, dry eyes (a condition called ‘Sicca syndrome/Sjogren’s syndrome) or intolerance to gluten in wheat (coeliac disease) 51. Professor Hirschfield described PBC as being like a ‘necklace of white cells around small bile ducts’ whereas PSC is like ‘concrete around the bile ducts.’
Watch Professor Hirschfield’s June 2014 presentation from 3m24: