IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy

Abstract EN

IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs.

When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis.

A 74-year-old male complained of dysphagia and abdominal pain.

Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis.

Liver biopsy showed storiform fibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal tract.

Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%.

Eight months earlier, an inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells.

Hot spots revealed 593 IgG4-positive cells and an IgG4/IgG ratio of 92%.

The serum IgG4 of the patient was elevated nearly 10 times upper limit of normal.

The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made.

There was good response to treatment with prednisolone and azathioprine.

The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult.

Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease.