Understanding autoimmune pancreatitis: Clinical features, management challenges, and association with malignancies

Core Tip

Core Tip: Autoimmune pancreatitis (AIP) is a unique form of pancreatitis, categorized into AIP-1 and AIP-2, with distinct clinical characteristics and associations. AIP-1, linked to elevated immunoglobulin G4 (IgG4) levels, exhibits higher relapse rates and predominantly affects older males, while AIP-2 is less common and associated with inflammatory bowel disease. Recognized as a manifestation of IgG4-related systemic disease, AIP poses an increased risk of malignancies, especially gastric, colorectal, and bladder cancers. Despite ongoing debates about the association with pancreatic cancer, careful diagnostic evaluation, including biopsy and response to steroids, is crucial for informed decision-making regarding surgery. AIP patients may have concurrent PanINs but generally experience better long-term outcomes compared to pancreatic cancer patients. Steroids remain the primary treatment, and emerging biomarkers like interferon alpha and interleukin-33 offer promising avenues for monitoring and managing AIP.