Gut Liver.  2013 Sep;7(5):616-620.

Immunoglobulin G4-Related Disease Mimicking Unresectable Gallbladder Cancer

Affiliations
  • 1Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 2Department of Internal Medicine and Liver Research Institute, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. gidoctor@snuh.org
  • 3Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 4Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

Abstract

Immunoglobulin G4 (IgG4)-related disease is a novel disease entity that can involve diverse organs, causing specific diseases, including autoimmune pancreatitis, sclerosing cholangitis, cholecystitis, inflammatory aortic aneurysm, and inflammatory pseudotumor. IgG4-related disease is characterized by elevated serum IgG4 concentrations, abundant IgG4 lymphoplasmacytic infiltration, and dramatic steroid responses. It is clinically important to differentiate this rare disease from primary sclerosing cholangitis and cholangiocarcinoma, because the treatment and prognosis of these two diseases are completely different. However, the preoperative diagnosis is challenging, and the disease is frequently misdiagnosed. If the serum level of IgG4 is within the normal range, the diagnosis of IgG4-related disease is more difficult. This article reports on a 59-year-old man with IgG4-related disease mimicking unresectable gallbladder cancer with normal serum IgG4 concentrations.

Keyword

Immunoglobuin G; Cholangitis, sclerosing; Gallbladder neoplasms; Cholecystitis

MeSH Terms

Aortic Aneurysm
Cholangiocarcinoma
Cholangitis, Sclerosing
Cholecystitis
Gallbladder
Gallbladder Neoplasms
Granuloma, Plasma Cell
Humans
Immunoglobulin G
Immunoglobulins
Middle Aged
Pancreatitis
Prognosis
Rare Diseases
Reference Values
Cholangitis, Sclerosing
Immunoglobulin G
Immunoglobulins
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