Review Article

IgG4 Related Sclerosing Cholangitis

Table 1

HISORt criteria for IgG4 associated cholangitis [26].

FeatureCharacteristics

Histology of bile ductLymphoplasmacytic sclerosing cholangitis on resection specimens (lymphoplasmacytic infiltrate with >10 IgG4-positive cells/high powered field within and around bile ducts with associated obliterative phlebitis and storiform fibrosis)

Imaging of bile duct(i) One stricture or more strictures involving intrahepatic, proximal extrahepatic, or intrapancreatic bile ducts
(ii) Fleeting/migrating biliary strictures

SerologyIncreased levels of serum IgG4

Other organs involvement pancreas, retroperitoneal fibrosis, renal lesion, and salivary/lacrimal gland

Response to steroid therapyNormalization of liver enzymes or resolution of stricture (radiologically)