Table 7.

Differences Between PSC and IgG4-SC

	PSC	IgG4-SC
Age	25–45yrs	65yrs
Male Gender	65%	80%
Association with IBD	Present	Absent
Other organ involvement	No	Pancreas frequently involved
p-ANCA	Positive	Less common
Elevated serum IgG4	7–9%	70%
Histology	Ductopenia Periductal concentric fibrosis	Abundant IgG4 + plasma cells Periportal fibroinflammatory nodules
Cholangiogram	Multifocal “beaded”; pruned tree appearance of intrahepatic and extrahepatic ducts	Segmental, long strictures with prestenotic dilatation, distal CBD involvement, pancreatic duct involvement
Response to Steroid	No	Yes

PSC –primary sclerosing cholangitis; IgG4-SC – IgG4 related sclerosing cholangitis