BACKGROUND:?Autoimmune?pancreatitis?(AIP)?is?increasingly?recognized?as?a?unique?subtype?of?pancreatitis.?This?study?aimed?to?analyze?the?diagnosis?and?treatment?of?AIP?patients?from?a?tertiary?care?center?in?China.METHODS:?One?hundred?patients?with?AIP?who?had?been?treated?from?January?2005?to?December?2012?in?our?hospital?were?enrolled?in?this?study.?We?retrospectively?reviewed?the?data?of?clinical?manifestations,?laboratory?tests,?imaging?examinations,?pathological?examinations,?treatment?and?outcomes?of?the?patients.RESULTS:?The?median?age?of?the?patients?at?onset?was?57?years?(range?23-82)?with?a?male?to?female?ratio?of?8.1:1.?The?common?manifestations?of?the?patients?included?obstructive?jaundice?(49?patients,?49.0%),?abdominal?pain?(30,?30.0%),?and?acute?pancreatitis?(11,?11.0%).?Biliary?involvement?was?one?of?the?most? extrapancreatic? manifestations? (64,? 64.0%).? Fifty-six?(56.0%)?and?43?(43.0%)?patients?were?classiifed?into?focal-type?and?diffuse-type?respectively?according?to?the?imaging?examinations.?The?levels?of?serum?IgG?and?IgG4?were?elevated?in?69.4%?(43/62)?and?92.0%?(69/75)?patients.?Pathological?analysis?of?specimens?from?27?patients?supported?the?diagnosis?of?lymphoplasmacytic?sclerosing?pancreatitis,?and?marked?(>10?cells/HPF)?IgG4?positive?cells?were?found?in?20?(74.1%)?patients.?Steroid?treatment?and?surgery?as?the?main?initial?treatments?were?given?to?41?(41.0%)?and?28?(28.0%)?patients,?respectively.?The?remission?rate?after?the?initial?treatment?was?85.0%.?Steroid?was?given?as?the?treatment?after?relapse?in?most?of?the?patients?and?the?total?remission?rate?at?the?end?of?follow-up?was?96.0%. CONCLUSIONS:?Clinical?manifestations,?laboratory?tests,?imaging?and?pathology?examinations?in?combination?could?increase?the?diagnostic?accuracy?of?AIP.?Steroid?treatment?with?an?initial?dose?of?30?or?40?mg?prednisone?is?effective?and?safe?in?most?patients?with?AIP.