Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibroinflammatory disease (IgG4-related systemic disease) in which affected organs demonstrate dense lymphoplasmacytic infiltration with abundant IgG4-positive cells. The diagnosis of AIP and its differentiation from pancreatic cancer, its main differential diagnosis, remains a clinical challenge. The five cardinal features of AIP are characteristic histology, imaging, and serology; other organ involvement; and response to steroid therapy. Recent advances in our understanding of these features have resulted in enhanced recognition and diagnosis of this benign disease. This in turn has resulted in the avoidance of unnecessary surgical procedures for suspected malignancy. This article reviews recent updates in the diagnosis and treatment of autoimmune pancreatitis.
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