IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions\nin different organs. When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis. A 74-\nyear-old male complained of dysphagia and abdominal pain. Endoscopic retrograde cholangiography and magnetic resonance\ncholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis. Liver biopsy showed storiform\nfibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal\ntract. Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%. Eight months earlier,\nan inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells. Hot spots revealed\n593 IgG4-positive cells and an IgG4/IgG ratio of 92%.The serumIgG4 of the patient was elevated nearly 10 times upper limit of\nnormal. The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was\ngood response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from\nprimary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, butwe\ndescribe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features\nof this disease.
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