Reducing relapse through maintenance steroid treatment can decrease the cancer risk in patients with IgG4-sclerosing cholangitis: Based on a Japanese nationwide study
Reducing relapse through maintenance steroid treatment can decrease the cancer risk in patients with IgG4-sclerosing cholangitis: Based on a Japanese nationwide study
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DOI：
10.1111/jgh.16066
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发表时间：
2023-01-10
影响因子：
4.1
通讯作者：
Okazaki, Kazuichi
中科院分区：
文献类型：
--
作者：
Kubota, Kensuke;Kamisawa, Terumi;Okazaki, Kazuichi
IgG 4相关性硬化性胆管炎（IgG 4-SC）被认为是一种良性类固醇反应性疾病;然而，对IgG 4-SC患者发生癌症的风险以及如何应对这种风险知之甚少。设计我们进行了一项回顾性分析的数据，924例患者的IgG 4-SC从日本全国范围内的调查。对这些患者的发病率、恶性肿瘤类型和恶性肿瘤风险进行了检查。然后，计算IgG 4-SC患者中癌症的标准化发病比（SIR）。结果19.7%（182/924）的患者复发，15%（139/924）的患者发生癌变。多变量分析仅将复发确定为癌症发展的独立风险因素。在这些胰胆管癌患者中，大多数在IgG 4-SC诊断后8年内发生癌症，IgG 4-SC诊断后癌症的SIR为12.68（95%置信区间[CI] 6.89-8.79）。胆管癌和胰腺癌的SIR分别为27.35和18.43。接受维持类固醇治疗（MST）组的累积生存率明显优于未接受维持类固醇治疗组;因此，MST影响这些患者的预后。结论胰腺癌和胆道癌的危险性最高。由于癌症风险升高，诊断后的监测和预防复发的管理对于IgG 4-SC患者降低癌症发展风险非常重要。
ObjectiveIgG4-related sclerosing cholangitis (IgG4-SC) is recognized as a benign steroid-responsive disease; however, little is known about the risk of development of cancer in patients with IgG4-SC and about how to counter this risk. DesignWe conducted a retrospective review of the data of 924 patients with IgG4-SC selected from a Japanese nationwide survey. The incidence, type of malignancy, and risk of malignancy in these patients were examined. Then, the standardized incidence ratio (SIR) of cancer in patients with IgG4-SC was calculated. ResultsRelapse was recognized in 19.7% (182/924) of patients, and cancer development was noted in 15% (139/924) of patients. Multivariate analysis identified only relapse as an independent risk factor for the development of cancer. In most of these patients with pancreato-biliary cancer, the cancer developed within 8 years after the diagnosis of IgG4-SC. The SIR for cancer after the diagnosis of IgG4-SC was 12.68 (95% confidence interval [CI] 6.89-8.79). The SIRs of cancers involving the biliary system and pancreas were 27.35 and 18.43, respectively. The cumulative survival rate was significantly better in the group that received maintenance steroid treatment (MST) than in the group that did not; thus, MST influenced the prognosis of these patients. ConclusionAmong the cancers, the risk of pancreatic and biliary cancers is the highest in these patients. Because of the elevated cancer risk, surveillance after the diagnosis and management to prevent relapse are important in patients with IgG4-SC to reduce the risk of development of cancer.