IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

IgG4-related sclerosing cholangitis.

Extensive Metastatic Cholangiocarcinoma Associated With IgG4-Related Sclerosing Cholangitis Misdiagnosed as Isolated IgG4-Related Sclerosing Cholangitis

As cholangiographic features of IgG4-related sclerosing cholangitis (IgG4-SC) resemble those of cholangiocarcinoma, it is highly confusing between the 2 conditions on the basis of cholangiographic findings. This study presents a case of extensive metastatic cholangiocarcinoma with IgG4-SC misdiagnosed as isolated IgG4-SC, and reviews recent studies of the 2 diseases.A 56-year-old man with no fa...

IgG4-related sclerosing cholangitis: liver biopsy findings

IgG4-related autoimmune disease is a recently recognized clinical entity associated with autoimmune pancreatitis, various systemic extrapancreatic lesions and an elevated serum IgG4 level. IgG4-related sclerosing cholangitis (IgG4-SC) is one of the extrapancreatic manifestations of this entity, which needs to be differentiated from primary sclerosing cholangitis (PSC) as these two diseases appe...

IgG4-negative autoimmune pancreatitis with sclerosing cholangitis and colitis: possible association with primary sclerosing cholangitis?

We report a case of autoimmune pancreatitis (AIP) with cholangiography and histopathology showing features characteristic of primary sclerosing cholangitis (PSC) and colitis. A 55-year-old previously-healthy man was diagnosed with anti-nuclear antibody (ANA)-positive AIP according to the finding of serum biochemistry, abdominal US (ultrasonography), CT (computed tomography) and ERCP (endoscopic...

Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disorder of the biliary system that results in progressive fibrosis and strictures of the intrahepatic and extrahepatic bile ducts. Typical radiological changes on retrograde cholangiography are shown in Figure 1. These strictures cause cholestatic liver disease that may progress to end-stage cirrhosis. The disorder has a strong ass...