Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

Abstract

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

Keywords: IgG4-related disease; cholangiocarcinoma; common bile duct; jaundice; prednisone; sclerosing cholangitis.

Figure 1.

Magnetic resonance cholangiopancreatography shows a mass in the common bile duct suggestive of bile duct carcinoma.

Figure 2.

Computed tomography of the liver and gallbladder shows nodular soft tissue density in the upper part of the common bile duct, which measures 12 × 15 mm, swelling of the upper liver, and dilation of the internal bile duct.

Figure 3.

Histology using hematoxylin and eosin staining shows extensive fibrotic tissue and infiltration of lymphocytes. Magnification, ×40.

Figure 4.

Histology using hematoxylin and eosin staining shows obliterative venulitis (arrow). Magnification, × 100.

Figure 5.

Immunohistochemistry shows a large number of immunoglobulin (Ig) G4-positive plasma cells infiltrating the tissue. The IgG4+/IgG+ ratio is approximately 50% to 60%. Magnification, × 200.