Abstract：
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.
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.
摘要：
背景：在自身免疫性胰腺炎（AIP）和IgG4相关性硬化性胆管炎（IgG4-SC）患者中，胰胆管癌的风险和预后仍不清楚。因此,我们回顾性调查了AIP和IgG4-SC患者的胰胆管癌风险和预后.
方法：调查了1998年至2022年七个中心的AIP和IgG4-SC患者。评估了以下数据：（1）在观察期间诊断的癌症数量和胰胆管和其他癌症的标准化发生率（SIR），以及（2）使用标准化死亡率（SMR）诊断AIP和IgG4-SC后的预后。
结果：本研究包括201例AIP和IgG4-SC患者。平均随访期为5.7年。诊断为胰腺癌7例，SIR为8.11（95%置信区间[CI]：7.29-9.13）。诊断为胆管癌3例，SIR为6.89（95%CI：6.20-7.75）。诊断为AIP和IgG4-SC后的SMR为4.03（95％CI：2.83-6.99）。
结论：自身免疫性胰腺炎和IgG4-SC患者与胰腺癌和胆管癌的高风险相关。患有AIP和IgG4-SC的患者在发生胰胆管癌时具有更差的预后。
方法：调查了1998年至2022年七个中心的AIP和IgG4-SC患者。评估了以下数据：（1）在观察期间诊断的癌症数量和胰胆管和其他癌症的标准化发生率（SIR），以及（2）使用标准化死亡率（SMR）诊断AIP和IgG4-SC后的预后。
结果：本研究包括201例AIP和IgG4-SC患者。平均随访期为5.7年。诊断为胰腺癌7例，SIR为8.11（95%置信区间[CI]：7.29-9.13）。诊断为胆管癌3例，SIR为6.89（95%CI：6.20-7.75）。诊断为AIP和IgG4-SC后的SMR为4.03（95％CI：2.83-6.99）。
结论：自身免疫性胰腺炎和IgG4-SC患者与胰腺癌和胆管癌的高风险相关。患有AIP和IgG4-SC的患者在发生胰胆管癌时具有更差的预后。
