目的:分析总结2009年至2018年我国自身免疫性胰腺炎(AIP)患者的临床特征。方法:以“自身免疫性胰腺炎、免疫性胰腺炎、IgG相关胰腺炎”为检索词，分别在中国知网、万方和维普数据库检索发表于2009年1月至2018年12月间的文献，最终纳入文献110篇，共计2 364例AIP患者，分析总结AIP患者的性别、年龄、临床特征、实验室检查结果、影像学表现、治疗方式及预后。结果:2 364例AIP患者中男性1 777例，女性587例，平均年龄58岁。主要临床表现为黄疸(61.31%)、腹痛(52.45%)、腹胀不适(34.78%)、体重减轻(22.62%)；50.13%患者有胰腺外表现。血清学检查主要表现为IgG4(74.14%)、CRP(73.68%)、IgG(71.41%)、血沉(67.34%)、总胆红素(62.44%)、ALT和AST(50.83%)及CA19-9(43.85%)水平明显升高，类风湿因子阳性(42.04%)。影像学检查表现为胰腺弥漫性肿大占46.87%，局限性肿大占21.87%。病理学主要表现为胰腺淋巴细胞和浆细胞浸润，腺泡破坏及萎缩，纤维组织增生及IgG4免疫组织化学阳性。282例(11.92%)患者被误诊，70.12%患者激素治疗有效。结论:我国AIP好发于男性，常伴有胰腺外表现，影像学检查可协助诊断，但误诊率较高。临床医师需提高对AIP的诊治意识和水平，减少漏诊误诊。“,”Objective:To analyze and summarize the clinical characteristics of autoimmune pancreatitis (AIP) patients in China from 2009 to 2018.Methods:Studies published from January 2009 to December 2018 were searched in CNKI, Wanfang and VIP databases by using “ autoimmune pancreatitis” , “ immune pancreatitis” and “ IgG related pancreatitis” as search items, respectively. One hundred and ten articles were finally included, and a total of 2 364 AIP patients were involved. The gender, age, clinical manifestations, laboratory tests, radiological examination, treatment and prognosis of these AIP patients were analyzed and summarized.Results:Among the 2 364 AIP patients, there were 1 777 males and 587 females, with an average age of 58 years. The main clinical manifestations were jaundice(61.31%), abdominal pain(52.45%), abdominal discomfort(34.78%) and body weight loss(22.62%). 50.13% of the patients suffered from extrapancreatic manifestations. Serological examination detected significantly increased levels of IgG4 (74.14%), CRP (73.68%), IgG (71.41%), erythrocyte sedimentation (67.34%), total bilirubin (62.44%), ALT/AST(50.83%), CA19-9(43.85%) and positive rheumatoid factor (42.04%). Pancreatic diffuse (46.87%) and regional enlargement (21.87%) was found in AIP patients by imaging examination. The pancreatic pathological manifestations mainly included lymphoplasmic cell infiltration, pancreatic acinar destruction and atrophy, fibrous tissue proliferation, and positive IgG4 deposition by immunohistochemistry. 282 patients (11.92%) were misdiagnosed, while 70.12% of AIP patients were attenuated by hormones therapies.Conclusions:AIP, which is more common in male in China, is often accompanied by extrapancreatic manifestations. Although imaging examinations were helpful for diagnosis, the misdiagnosis rate remains high. Clinicians need to raise awareness, enhance the ability to diagnose and treat AIP, thus reducing misdiagnosis.