Immunoglobulin G4-related sclerosing cholangitis

Immunoglobulin (Ig)G4-related sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related diseases (IgG4-RD); a subgroup of SC defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis. IgG4-SC is clinically characterized by the (a) chronic elevation of cholestatic enzyme levels, (b) significant elevation of serum IgG4 levels, (c) diffuse or segmental narrowing of intra and/or extra hepatic bile ducts with thickening of the bile duct wall in imaging studies, (d) marked lymphoplasmacytic and IgG4-positive plasma cell infiltration and fibrosis in histology, (e) presence of IgG4-RD in other organs, mainly involving autoimmune pancreatitis, and (f) excellent response to corticosteroids. The diagnosis of IgG4-SC is based on a combination of these findings. Although the IgG4-SC diagnosis is different from that of primary sclerosing cholangitis (PSC) or biliary and pancreatic malignancies, it is extremely important to diagnose or suspect IgG4-SC appropriately; as the incorrect diagnosis of PSC or malignant diseases may lead to the progression of fibrosis in patients due to untreated chronic cholestasis, or to unnecessary major surgical resections. Although its etiology remains unclear, recent studies of IgG4-SC have attempted to clarify the roles of the IgG4 molecule and novel autoantibodies detected in patients with IgG4-SC.