IgG4-associated cholangitis with cholangiocarcinoma
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Autoimmune pancreatitis (AIP) was first described by Sarles and coauthors  as a primary inflammatory sclerosis of the pancreas and is currently subclassified into two clinical entities named type 1 and type 2 AIP . Clinical features of type 1 AIP include elevated IgG4-levels in the serum and excellent response to steroids, whereas in type 2 AIP, IgG4 levels are not elevated. Histologic criteria for type 1 AIP include periductal lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative venulitis, whereas the hallmark of type 2 AIP is the presence of epithelial granulocytic lesions. Extrapancreatic disease occurs in more than 45% of patients with AIP, and especially intra- and extrahepatic biliary tree involvement has been described as a peculiar form of sclerosing cholangitis [3, 4, 5]. Less frequently, the disease may only involve the intra- and/or extrahepatic bile ducts, then termed IgG4-associated cholangitis (IAC) . Currently, IAC and AIP are seen as...
KeywordsBile Duct Cholangiocarcinoma Primary Sclerosing Cholangitis Extrahepatic Bile Duct Intrahepatic Cholangiocarcinoma
Magnetic resonance cholangiopancreatography
Primary sclerosing cholangitis
BKS was supported by a stipend of the Olympia-Morata program of the Medical Faculty of Heidelberg University.
The authors declare that no financial or other conflict of interest exists in relation to the content of the article.