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. 2013 Sep;27(9):523–530. doi: 10.1155/2013/180461

TABLE 6.

Differences between primary sclerosing cholangitis and immunoglobulin (Ig)G4 cholangiopathy

Feature Primary sclerosing cholangitis IgG4 cholangiopathy or autoimmune cholangiopathy
Age Young to middle age Older
Obstructive jaundice Marked Less severe
Biliary strictures Band-like strictures with beaded and pruned tree appearance Segmental strictures (distal CBD)
Associated autoimmune pancreatitis No Yes
IgG4-related diseases No Yes
IBD association Yes Rarely associated with type 2 AIP
Risk of cholangiocarcinoma Yes Rare (with limited data), can mimic

Adapted from references 57 and 67. AIP Autoimmune pancreatitis; CBD Common bile duct; IBD Inflammatory bowel disease