Table 3 Differential diagnosis between primary biliary cholangitis, primary sclerosing cholangitis and IgG4-related sclerosing cholangitis

Vulnerable populations

Middle-aged woman

Young-aged man

Elderly patients

Possible risk factors

Synergy of multiple factors, including environmental, genetic/epigenetic, and immunological factors

Close relationship with HLA antigens; the intestinal microbiome is key to immune response initiation

Poorly understood

AMA

Presence; highly sensitive and specific for PBC diagnosis; or presence of PBC-specific autoantibodies such as sp100 or gp210 if AMA is negative

Absence

Absence

MRCP features

Segmental small bile duct dilatation, stenosis or poor visualization, mostly involving the intrahepatic secondary bile ducts

Beaded appearance of the intrahepatic and/or extrahepatic bile ducts in the early stages; “pruned-tree” appearance in more advanced stages; the cystic duct, gallbladder, and pancreatic duct may also be affected

Isolated or continuous stricture of the bile duct and upstream dilatation, most commonly affect the intrapancreatic segment of the common bile duct

Concomitant diseases

Rarely combined with inflammatory bowel disease

Often combined with inflammatory bowel disease

Often accompanied by autoimmune pancreatitis, other organs and parts of the body may also be involved