IgG4 related sclerosing cholangitis (IgG4-SC) is a chronic cholestatic autoimmune liver disease, characterized by obstruction of the biliary tract due to IgG4-positive plasma cell infiltrations associated with fibrosis, phlebitis and presence of eosinophils. The diagnosis of the disease is supported by elevation in serum IgG4, radiological imaging and histology. In most of the cases IgG4-SC is associated with a wide spectrum of symptoms and manifestations summarized by the definition of IgG4-related disease (IgG4-RD). When the IgG4-SC causes an interruption of the biliary tract, differential diagnosis with cholangiocarcinoma may become challenging, due to the anatomical difficulty to get an appropriate histological pattern of the disease. Here we are describing a case of a male patient admitted to our hospital for jaundice. The stenosis of biliary ducts shown by radiologic imaging suggested Cholangiocarcinoma but, in the patient, IgG4-SC was definitely diagnosed.

Sclerosing cholangitis may mimic radiological pattern of cholangiocarcinoma: Differential diagnosis and review of literature

Isaia, Ivan
Conceptualization
;
Colaci, Michele
Conceptualization
;
Malatino, Lorenzo
Conceptualization
2022

Abstract

IgG4 related sclerosing cholangitis (IgG4-SC) is a chronic cholestatic autoimmune liver disease, characterized by obstruction of the biliary tract due to IgG4-positive plasma cell infiltrations associated with fibrosis, phlebitis and presence of eosinophils. The diagnosis of the disease is supported by elevation in serum IgG4, radiological imaging and histology. In most of the cases IgG4-SC is associated with a wide spectrum of symptoms and manifestations summarized by the definition of IgG4-related disease (IgG4-RD). When the IgG4-SC causes an interruption of the biliary tract, differential diagnosis with cholangiocarcinoma may become challenging, due to the anatomical difficulty to get an appropriate histological pattern of the disease. Here we are describing a case of a male patient admitted to our hospital for jaundice. The stenosis of biliary ducts shown by radiologic imaging suggested Cholangiocarcinoma but, in the patient, IgG4-SC was definitely diagnosed.
Autoimmune
Cholangiocarcinoma
IgG4-related disease
IgG4-sclerosing cholangitis
Bile Ducts, Intrahepatic
Diagnosis, Differential
Humans
Immunoglobulin G
Male
Bile Duct Neoplasms
Cholangiocarcinoma
Cholangitis, Sclerosing
Cholestasis
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11769/537504
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