doiSerbia

A case of autoimmune cholangitis misdiagnosed for cholangiocarcinoma: How to avoid unnecessary surgical intervention?

Ignjatović Igor I. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade)
Matić Slavko V. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade + School of Medicine, Belgrade)
Dugalić Vladimir D. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade + School of Medicine, Belgrade)
Knežević Đorđe M. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade + School of Medicine, Belgrade)
Micev Marjan T. (School of Medicine, Belgrade + Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Pathology, Belgrade)
Bogdanović Marko D. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade)
Knežević Srbislav M. (Clinical Center of Serbia, Clinic for Digestive Surgery - First Surgical Clinic, Department of Hepatobiliary and Pancreatic Surgery, Belgrade + School of Medicine, Belgrade)

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.

Keywords: autoimmune cholangitis, cholangiocarcinoma, differential diagnosis