Table 7.
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