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Background: The risk and prognosis of pancreatobiliary cancer and in patients with autoimmune pancreatitis (AIP) and IgG4‐related sclerosing cholangitis (IgG4‐SC) remain unclear. Therefore, we retrospectively investigated the risk of pancreatobiliary cancer and prognosis in patients with AIP and IgG4‐SC. Methods: Patients with AIP and IgG4‐SC at seven centers between 1998 and 2022 were investigated. The following data were evaluated: (1) the number of cancers diagnosed and standardized incidence ratio (SIR) for pancreatobiliary and other cancers during the observational period and (2) prognosis after diagnosis of AIP and IgG4‐SC using standardized mortality ratio (SMR). Results: This study included 201 patients with AIP and IgG4‐SC. The mean follow‐up period was 5.7 years. Seven cases of pancreatic cancer were diagnosed, and the SIR was 8.11 (95% confidence interval [CI]: 7.29–9.13). Three cases of bile duct cancer were diagnosed, and the SIR was 6.89 (95% CI: 6.20–7.75). The SMR after the diagnosis of AIP and IgG4‐SC in cases that developed pancreatobiliary cancer were 4.03 (95% CI: 2.83–6.99). Conclusions: Patients with autoimmune pancreatitis and IgG4‐SC were associated with a high risk of pancreatic and bile duct cancer. Patients with AIP and IgG4‐SC have a worse prognosis when they develop pancreatobiliary cancer. [ABSTRACT FROM AUTHOR]

Background: The risk and prognosis of pancreatobiliary cancer and in patients with autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC) remain unclear. Therefore, we retrospectively investigated the risk of pancreatobiliary cancer and prognosis in patients with AIP and IgG4-SC., Methods: Patients with AIP and IgG4-SC at seven centers between 1998 and 2022 were investigated. The following data were evaluated: (1) the number of cancers diagnosed and standardized incidence ratio (SIR) for pancreatobiliary and other cancers during the observational period and (2) prognosis after diagnosis of AIP and IgG4-SC using standardized mortality ratio (SMR)., Results: This study included 201 patients with AIP and IgG4-SC. The mean follow-up period was 5.7 years. Seven cases of pancreatic cancer were diagnosed, and the SIR was 8.11 (95% confidence interval [CI]: 7.29-9.13). Three cases of bile duct cancer were diagnosed, and the SIR was 6.89 (95% CI: 6.20-7.75). The SMR after the diagnosis of AIP and IgG4-SC in cases that developed pancreatobiliary cancer were 4.03 (95% CI: 2.83-6.99)., Conclusions: Patients with autoimmune pancreatitis and IgG4-SC were associated with a high risk of pancreatic and bile duct cancer. Patients with AIP and IgG4-SC have a worse prognosis when they develop pancreatobiliary cancer., (© 2023 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)