Introduction: IgG4-related disease is a recently recognized fibroinflammatory condition that can involve multiple organs. We report a case with pancreatic, biliary, and renal involvement. Observation: This was a 38-year-old patient presenting with jaundice associated with generalized pruritus and weight loss. He had a history of allergic rhinitis. Physical examination revealed jaundice and excoriations. Laboratory tests showed cholestasis and cytolysis. The serum IgG4 level was 3.63 g/L. Abdominal computed tomography (CT) and MR cholangiopancreatography demonstrated features consistent with autoimmune disease with pancreatic, biliary, and renal involvement. The clinical course was favorable under long-term corticosteroid therapy. Conclusion: IgG4-related disease is rare but probably underdiagnosed in Africa. It often has a favorable course with appropriately administered corticosteroid therapy.
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