Isolated IgG4-related sclerosing cholangitis: a report of 9 cases

Extrahepatic bile ducts are the most commonly involved extrapancreatic organ site in patients with type 1 autoimmune pancreatitis. IgG4-related sclerosing cholangitis (IgG4-SC) alone, without evidence of pancreatic or other organ involvement, is uncommon and is difficult to distinguish from cholangiocarcinoma preoperatively. We describe 9 patients with isolated IgG4-SC over an approximate 10-year period, each clinically suspected to have...