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Background Autoimmune pancreatitis (ALP) is a chronic inflammatory disease of pancreas.We evaluated the clinical manifestations,imaging,and histological presentations of AlP in Chinese patients,and investigated the roles of immunoglobulin E (IgE) and allergic diseases in the diagnosis and pathogenesis of AIP.Methods The clinical records of 22 patients diagnosed with AlP were reviewed and analyzed.All patients with AlP fulfilled the 2006 revised diagnostic criteria proposed by Japan Pancreas Society or the Korean Criteria for AIP.Results Half (11/22) of AlP patients had allergic diseases.Twenty-one patients had elevated serum IgE levels,and 14 patients had IgE levels more than 3 times that of normal.There were no significant differences between the patients with higher or lower IgE,with or without allergic disease,in clinical features,laboratory tests,diffuse or focal lesions,or the choice of treatment methods; however,more complaints of body weight loss were observed in patients with higher IgE levels.Patients with higher IgE levels and with allergic diseases were more likely to have onset in March,April,May,August,September,or October.IgE levels decreased after therapy,but increased again during recurrence.Increased number of mast cells was found in the pancreatic tissue in AIP.Conclusions IgE maybe a useful marker for monitoring therapeutic response and recurrence of AlP.Allergic processes may play an important role in the pathogenesis of AIP.