Introduction. Autoimmune cholangitis or immunoglobulin G4-associated
cholangitis (IAC) has been recently regarded as a new clinical and
histopathological entity and is a part of a complex autoimmune disorder -
IgG4-related systemic disease (ISD). ISD is an autoimmune disease with
multi-organic involvement, characterized with IgG4-positive plasmocytic
infiltration of various tissues and organs with a consequent sclerosis, which
responds well to steroid therapy. Most commonly affected organs are the
pancreas (autoimmune pancreatitis, [AIP]) and the common bile duct (IAC). IAC
and cholangiocarcinoma (CCA) share many clinical, laboratory and imaging
findings. Case Outline. We present a case of a 60-year-old male with a
biliary stricture of a common bile duct, which was clinically considered as a
bile duct carcinoma and treated surgically. Definite histopathological
findings and immunohistochemistry revealed profound chronic inflammation,
showing lymphoplasmacytic IgG-positive infiltration of a resected part of a
common bile duct, highly suggestive for the diagnosis of IAC. In addition,
postoperative IgG4 serum levels were also increased. Conclusion. It is of
primary clinical importance to make a difference between IAC and CCA, in
order to avoid unnecessary surgical intervention. Therefore, IAC should be
considered in differential diagnosis in similar cases.
Pancreaticobiliary Maljunction Is Associated with Common Bile Duct Carcinoma: A Meta-Analysis
