Gut Liver.  2013 Mar;7(2):234-238.

Differentiating Immunoglobulin G4-Related Sclerosing Cholangitis from Hilar Cholangiocarcinoma

Affiliations
  • 1Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp
  • 2Department of Endoscopy, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
  • 3Department of Surgery, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
  • 4Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan.

Abstract

BACKGROUND/AIMS
Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC.
METHODS
We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC.
RESULTS
In patients with hilar CC, obstructive jaundice was more frequent (p<0.01), serum total bilirubin levels were significantly higher (p<0.05), serum CA19-9 levels were significantly higher (p<0.01), and serum duke pancreatic monoclonal antigen type 2 levels were frequently elevated (p<0.05). However, in patients with IgG4-SC, the serum IgG (p<0.05) and IgG4 (p<0.01) levels were significantly higher and frequently elevated. The pancreas was enlarged in all IgG4-SC patients but only in 17% of hilar CC patients (p<0.01). Salivary and/or lacrimal gland swelling was detected in only 50% of IgG4-SC patients (p<0.01). Endoscopic retrograde cholangiography revealed that the hilar or hepatic duct was completely obstructed in 83% of hilar CC patients (p<0.01). Lower bile duct stenosis, apart from hilar bile duct stenosis, was more frequent in IgG4-SC patients (p<0.01). Bile duct wall thickening in areas without stenosis was more frequent in IgG4-SC patients (p<0.01).
CONCLUSIONS
An integrated diagnostic approach based on clinical, serological, imaging, and histological findings is necessary to differentiate IgG4-SC from hilar CC.

Keyword

Immunoglobulin G; IgG4-ralated sclerosing cholangitis; Hilar cholangiocarcinoma; Endoscopic retrograde cholangiopancreatography

MeSH Terms

Bile Ducts
Bilirubin
Cholangiocarcinoma
Cholangiography
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis, Sclerosing
Constriction, Pathologic
Hepatic Duct, Common
Humans
Immunoglobulin G
Immunoglobulins
Jaundice, Obstructive
Lacrimal Apparatus
Pancreas
Retrospective Studies
Bilirubin
Immunoglobulin G
Immunoglobulins
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