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BACKGROUND:?Autoimmune? pancreatitis? (AIP)? is? a? rare?form?of?chronic?pancreatitis?with?a?discrete?pathophysiology,?occasional?diagnostic?radiological?findings,?and?characteristic?histological? features.? Its? etiology? and? pathogenesis? are?still? under? investigation,? especially? during? the? last? decade.?Another?aspect?of?interest?is?the?attempt?to?establish?specific?criteria? for? the? differential? diagnosis? between? autoimmune?pancreatitis?and?pancreatic?cancer,?entities?that?are?frequently?indistinguishable. DATA?SOURCES:?An?extensive?search?of?the?PubMed?database?was?performed?with?emphasis?on?articles?about?the?differential?diagnosis? between? autoimmune? pancreatitis? and? pancreatic?cancer?up?to?the?present. RESULTS:?The?most?interesting?outcome?of?recent?research?is?the?theory? that? autoimmune?pancreatitis?and? its?various?extra-pancreatic? manifestations? represent? a? systemic? fibro-inflammatory? process? called? IgG4-related? systemic? disease.?The?diagnostic?criteria?proposed?by?the?Japanese?Pancreatic?Society,? the? more? expanded? HISORt? criteria,? the? new?definitions? of? histological? types,? and? the? new? guidelines?of? the? International? Association? of? Pancreatology? help? to?establish?the?diagnosis?of?the?disease?types. CONCLUSION:?The? valuable? help? of? the? proposed? criteria? for?the?differential?diagnosis?between?autoimmune?pancreatitis?and?pancreatic? cancer? may? lead? to? avoidance? of? pointless? surgical?treatments?and?increased?patient?morbidity.