Abstract: Objective The aim of this study was to summarize clinical feature of patients with IgG4-related sclerosing cholangitis (IgG4-SC) and concomitant autoimmune pancreatitis (AIP). Methods 28 patients with AIP and 19 patients with AIP and concomitant IgG4-SC were encountered in our hospital between November 2020 and April 2023, and all underwent liver biopsies and abdominal CT scan. Serum IgG and IgG4 levels were detected by ELISA, and IgG4-RD activity responder index (RI) were calculated. Patients were treated by prednisone and/or ursodeoxycholic acid (UDCA). Results Percentage of concomitant diabetes, involved organ and RI in patients with AIP and IgG4-SC were 68.4%, 3.0(2.5, 4.5) and 12.5(12.0, 14.5), all significant higher than [35.7%, 2.0(1.0, 2.5) and 7.0(5.0, 8.0), respectively, P<0.05] in patients with AIP; incidence of abdominal pain in patients with AIP and IgG4-SC was 73.7%, much higher than 42.9%(P<0.05) in patients with AIP; serum GGT, ALP, IgG, amylase and lipase levels in patients with AIP and IgG4-SC were 574.7(76.8, 965.2)U/L, 438.0(274.4, 638.7)U/L, 141.6(134.7, 208.5)g/L, 38.5(29.7, 79.2)U/L and 49.3(36.1, 108.5)U/L, all much higher than [298.8(37.5, 685.3)U/L, 168.5(109.4, 374.3)U/L, 45.3(30.5, 69.4)g/L, 16.6(10.6, 28.0)U/L and 14.5(8.5, 25.8)U/L, respectively, P<0.05] in patients with AIP; percentages of diffuse pancreatic enlargement, pancreatic stones or calcification and intrahepatic bile duct wall thickening in patients with AIP and IgG4-SC were 78.9%, 15.8% and 15.8%, all much higher than 46.4%, 3.6% and 3.6% (P<0.05) in patients with AIP. Conclusion Autoimmune pancreatitis and concomitant IgG4-SC might be clinically sophisticated, and clinicians should assess the disease carefully. We recommend prednisone and UDCA treatment for them.

Key words: IgG4-related sclerosing cholangitis, Autoimmune pancreatitis, Clinical feature, Prognosis