Abstract

Autoimmune pancreatitis (AIP) is a steroid-responsive fibroinflammatory disorder with two clinically distinct subtypes known as type 1 autoimmune and type 2 autoimmune pancreatitis. Type 1 AIP is considered the pancreatic manifestation of Immunoglobulin G4-related disease (IgG4-RD); a systemic disease often presenting with other organ involvement. Advances in understanding the unique clinical presentation, imaging findings, histopathology and clinical course of this relatively uncommon disease has led to international consensus regarding diagnosis and treatment. While corticosteroids remain the mainstay of treatment, several emerging novel therapies have been explored primarily in the context or relapsing and refractory cases. It is important that AIP be differentiated from other pancreatic conditions, especially since the clinical presentation may mimic pancreaticobiliary malignancies. Early recognition of this distinct entity and appropriate management can reduce the risk of complications and improve long-term outcomes.

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