Histopathological features of autoimmune pancreatitis

Autoimmune pancreatitis (AIP) is a chronic fibroinflammatory disease of the pancreas characterised by lymphoplasmacytic infiltrates, interstitial fibrosis, periductal inflammation and periphlebitis. Although first described more than four decades ago, it has not gained widespread attention until the 1990s when new insights into its aetiology, clinical presentation and management were discovered. Although nowadays widely accepted as a form of chronic pancreatitis, recent evidence suggests that AIP might not be confined to the pancreas but rather be an inflammatory pancreaticobiliary disease (autoimmune pancreatocholangitis, AIPC) with possible systemic involvement and association with other autoimmune disorders. This article reviews current concepts of AIP with special focus on the histopathological features of the disease.