Gaucher's disease in a patient with chronic active hepatitis

Gaucher's disease, an inherited metabolic disorder, may cause hepatic fibrosis, but hepatic inflammation does not occur as part of the disorder. The case of an 18-year-old girl of Ashkenazic Jewish ancestry with chronic active hepatitis and coexistent Gaucher's disease is presented. The clinical course of remissions and exacerbations of the disease activity was typical of "autoimmune" chronic active hepatitis and seemed unaffected by the coexistence of Gaucher's disease. Steroid and immunosuppressive treatment resulted in prompt resolution of the chronic hepatitis. Gaucher's disease results in B cell stimulation and polyclonal gammopathy, which may have contributed to the hypergammaglobulinemia associated with idiopathic ("autoimmune") chronic hepatitis in this case.