Objective To summarize the clinical characteristics, diagnosis and therapy of type 1 autoimmune pancreatitis (AIP). Methods Totally 9 patients diagnosed with type 1 AIP in Zhongshan Hospital from October 2012 to October 2018 were enrolled. Retrospective analysis of clinical manifestations, laboratory tests, imaging, histopathological examination, treatment and prognosis was conducted. Results Patients commonly presented jaundice, bloating, abdominal ache, both appetite and weight loss. The most common other organ involvements (OOI) were bile duct, lymph nodes, kidney and retroperitoneal tissue in order. All 9 cases had increased serum immunoglobulin G4 levels and 8 cases had 2 times higher than upper limits of normal levels. Upper abdominal MRI showed pancreatic enlargement in 7 cases, pancreatic mass in 2 cases and envelope-like edges in 3 cases. No histopathological diagnosis was obtained with tissues from fine needle aspiration in 2 cases. All the patients received steroid treatment with well results. Two cases who underwent endoscopic retrograde biliary drainage were found liver abscesses and common bile duct stone respectively. Recurrent cases still were improved after steroid retreatment or steroid combined with immune inhibitor usage. Conclusions Only less patients with type 1 AIP have typical imaging findings. Type 1 AIP needs to be diagnosed carefully and evaluated systematically. Treatment of type 1 AIP with steroid could be effective.
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