Abstract
IgG4-related disease is a phenotypically heterogeneous systemic autoimmune and inflammatory disease. The described phenotypes of this rare disease include (a) Pancreato-hepatobiliary disease, (b) head-and-neck limited disease, (c) retroperitoneal fibrosis and/or aortitis and (d) Mikulicz’s syndrome with systemic involvement. However, IgG4-related hepatopathy has not been well described in the literature with very few cases reported so far. We present a case of a 72 years old male who presented with itching, icterus and elevated liver function tests, and was diagnosed with IgG4-related hepatopathy based on histopathology. Patient had remarkable resolution of symptoms and laboratory abnormalities with corticosteroids and did not need additional immunosuppressive agents.