IgG4-associated cholangitis with cholangiocarcinoma

Excerpt

Autoimmune pancreatitis (AIP) was first described by Sarles and coauthors [1] as a primary inflammatory sclerosis of the pancreas and is currently subclassified into two clinical entities named type 1 and type 2 AIP [2]. 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 [3‐5]. Less frequently, the disease may only involve the intra- and/or extrahepatic bile ducts, then termed IgG4-associated cholangitis (IAC) [6]. Currently, IAC and AIP are seen as manifestations of a systemic IgG4-disease [6], often occurring together as autoimmune pancreatocholangitis (AIPC) [7]. While in primary sclerosing cholangitis (PSC), an association with malignancy, i.e., cholangiocarcinoma (CC), is well established with an incidence rate of 1.5% per year [8], an association of IAC/AIPC with invasive carcinoma of the biliary tree has not been demonstrated so far. Only recently, a case of biliary intraepithelial neoplasia in the common bile duct in the presence of bile duct affection of AIPC has been described [9]. Here, we demonstrate the first case of co-occurrence with a potentially correlated malignancy, i.e., intrahepatic CC. …