Epidemiology, comorbidities, treatments and outcomes of autoimmune liver diseases: A French nationwide study

IF 7.5 1区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY
Christophe Corpechot , Pierre Hornus , Mallory Cals , Pierre Rinder , Théo Marcille , Amina Malek , Karima Ben Belkacem , Farid Gaouar , Yasmina Chabane , Pierre-Antoine Corret , Paola Squarzoni , Pierre-Antoine Soret , Sara Lemoinne , Olivier Chazouillères , Angela Leburgue
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引用次数: 0

Abstract

Background & Aims

The epidemiology, clinical management, and prognosis of autoimmune liver diseases (AILDs) – including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) – vary according to geography and time. This study aimed to provide a comprehensive evaluation of the burden of AILDs in a Western European country.

Method

A nationwide retrospective study was performed using the French national health data system from 2009 to 2019. AIH and PBC were identified via ICD-10 codes, while PSC was defined using composite criteria. Prevalence, incidence, geographic patterns, comorbidities, treatments, liver transplant, and standardized mortality ratio were assessed.

Results

A total of 30,255 AILD cases were identified, representing 5% of chronic liver disease cases. The prevalence per 100,000 inhabitants was 14.9 for AIH, 15.0 for PBC, and 4.2 for PSC. Geographic variation was observed, with significant regional clustering of AIH and PBC cases. The incidence of AIH increased significantly over time, whereas that of PBC and PSC declined. Patients with AILDs exhibited higher rates of diabetes and all-cause malignancies compared to the general population. Ursodeoxycholic acid was underprescribed in PBC, while corticosteroids were frequently overused in both PBC and PSC, and ursodeoxycholic acid in AIH. Liver transplantation was performed four times more often in PSC than in either AIH or PBC. All AILDs were associated with elevated 10-year standardized mortality ratios: 1.80 for AIH, 1.74 for PBC, and 2.59 for PSC.

Conclusion

These findings confirm the rising incidence of AIH – but not PBC or PSC – the non-random geographic distribution of AIH and PBC, a higher risk of diabetes and cancer across all AILDs, and persistent excess mortality despite current treatment options.

Impact and implications

The true burden of autoimmune liver diseases (AILDs) remains inadequately characterized. In this extensive study conducted in France, based on health insurance and hospital records collected between 2009 and 2019, we characterized the epidemiology, comorbidities, treatments and outcomes of autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis. We confirm the rising incidence of AIH, the non-random geographic distribution of both autoimmune hepatitis and primary biliary cholangitis, the elevated risk of diabetes and cancer, and a persistently increased mortality across all AILDs – most notably in primary sclerosing cholangitis, where both excess mortality and the need for liver transplantation is higher than in other AILDs. These findings highlight the persistent gaps and unmet needs in the management of AILDs.

Abstract Image

自身免疫性肝病的流行病学、合并症、治疗和结局:一项法国全国性研究
背景和目的自身免疫性肝病(AILDs)——包括自身免疫性肝炎(AIH)、原发性胆道性胆管炎(PBC)和原发性硬化性胆管炎(PSC)——的流行病学、临床管理和预后因地域和时间而异。本研究旨在对一个西欧国家的艾滋病负担进行全面评估。方法采用2009 - 2019年法国国家卫生数据系统进行全国性回顾性研究。AIH和PBC通过ICD-10代码进行鉴定,而PSC使用复合标准进行定义。评估了患病率、发病率、地理分布、合并症、治疗、肝移植和标准化死亡率。结果共发现30255例AILD,占慢性肝病病例的5%。每10万居民中AIH患病率为14.9,PBC患病率为15.0,PSC患病率为4.2。观察到地理差异,AIH和PBC病例具有显著的区域聚集性。AIH的发病率随着时间的推移显著增加,而PBC和PSC的发病率则下降。与一般人群相比,aild患者表现出更高的糖尿病和全因恶性肿瘤发生率。熊去氧胆酸在PBC中使用不足,而皮质类固醇在PBC和PSC中经常被过度使用,熊去氧胆酸在AIH中被过度使用。肝移植在PSC患者中的应用是AIH或PBC患者的4倍。所有aild均与10年标准化死亡率升高相关:AIH为1.80,PBC为1.74,PSC为2.59。结论:这些发现证实了AIH的发病率上升,但PBC或PSC的发病率没有上升,AIH和PBC的非随机地理分布,所有aild中糖尿病和癌症的风险较高,尽管目前有治疗方案,但死亡率仍然过高。影响和意义自身免疫性肝病(AILDs)的真正负担仍然没有充分的特征。在法国进行的这项广泛的研究中,基于2009年至2019年收集的健康保险和医院记录,我们描述了自身免疫性肝炎、原发性胆道炎和原发性硬化性胆管炎的流行病学、合并症、治疗和结局。我们证实了AIH发病率的上升,自身免疫性肝炎和原发性胆道炎的非随机地理分布,糖尿病和癌症的风险升高,以及所有aild的死亡率持续上升-最明显的是原发性硬化性胆管炎,其死亡率和肝移植需求高于其他aild。这些发现突出了在艾滋病管理方面持续存在的差距和未满足的需求。
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来源期刊
JHEP Reports
JHEP Reports GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
12.40
自引率
2.40%
发文量
161
审稿时长
36 days
期刊介绍: JHEP Reports is an open access journal that is affiliated with the European Association for the Study of the Liver (EASL). It serves as a companion journal to the highly respected Journal of Hepatology. The primary objective of JHEP Reports is to publish original papers and reviews that contribute to the advancement of knowledge in the field of liver diseases. The journal covers a wide range of topics, including basic, translational, and clinical research. It also focuses on global issues in hepatology, with particular emphasis on areas such as clinical trials, novel diagnostics, precision medicine and therapeutics, cancer research, cellular and molecular studies, artificial intelligence, microbiome research, epidemiology, and cutting-edge technologies. In summary, JHEP Reports is dedicated to promoting scientific discoveries and innovations in liver diseases through the publication of high-quality research papers and reviews covering various aspects of hepatology.
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