Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.
Department of Medicine, Regional Hospital Horsens, Horsens, Denmark.
Liver Int. 2022 Nov;42(11):2466-2472. doi: 10.1111/liv.15382. Epub 2022 Aug 18.
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease associated with an increased prevalence of extrahepatic autoimmune diseases and an increased mortality compared with the general population. The contribution of extrahepatic autoimmune diseases to the increased mortality has not been clarified. Our aim was to determine the effect of extrahepatic autoimmune diseases on mortality in AIH patients.
This nationwide register-based cohort study included all Danish patients diagnosed with AIH between 1995 and 2019. We examined the presence of extrahepatic autoimmune diseases and compared the mortality between AIH patients with and without extrahepatic autoimmune diseases. We adjusted our analysis for age, sex, calendar year of AIH diagnosis, cirrhosis, cancer, chronic obstructive pulmonary disease and ischaemic heart disease.
We included 2479 AIH patients of whom 19.8% had one extrahepatic autoimmune disease and 3.3% had multiple. The adjusted 10-year cumulative mortality was 27.2% (95% confidence interval [CI]: 25.2-29.4) for patients with extrahepatic autoimmune diseases and 21.6% (95% CI: 19.9-23.6) for patients without. The adjusted mortality hazard ratio was 1.30 (95% CI: 1.12-1.52) for AIH patients with versus without extrahepatic autoimmune diseases; it was 1.25 (95% CI: 1.06-1.48) for patients with one extrahepatic autoimmune disease and 1.54 (95% CI: 1.15-2.05) for those with more than one.
Extrahepatic autoimmune diseases increased the mortality in patients with AIH. Patients with multiple extrahepatic autoimmune diseases had a higher mortality than patients with just one extrahepatic autoimmune disease.
自身免疫性肝炎（AIH）是一种慢性炎症性肝病，与肝外自身免疫性疾病的患病率增加和与普通人群相比死亡率增加有关。肝外自身免疫性疾病对死亡率的影响尚不清楚。我们的目的是确定 AIH 患者肝外自身免疫性疾病对死亡率的影响。
这是一项全国范围内基于登记的队列研究，纳入了 1995 年至 2019 年间所有被诊断为 AIH 的丹麦患者。我们检查了肝外自身免疫性疾病的存在情况，并比较了 AIH 患者有无肝外自身免疫性疾病的死亡率。我们调整了年龄、性别、AIH 诊断的日历年份、肝硬化、癌症、慢性阻塞性肺疾病和缺血性心脏病等因素的分析。
我们纳入了 2479 名 AIH 患者，其中 19.8%有 1 种肝外自身免疫性疾病，3.3%有多发性。有肝外自身免疫性疾病的患者 10 年累积死亡率为 27.2%（95%置信区间：25.2-29.4），无肝外自身免疫性疾病的患者为 21.6%（95%置信区间：19.9-23.6）。AIH 患者有肝外自身免疫性疾病的调整后死亡率风险比为 1.30（95%置信区间：1.12-1.52），有 1 种肝外自身免疫性疾病的为 1.25（95%置信区间：1.06-1.48），有多种的为 1.54（95%置信区间：1.15-2.05）。
肝外自身免疫性疾病增加了 AIH 患者的死亡率。患有多种肝外自身免疫性疾病的患者比仅患有 1 种肝外自身免疫性疾病的患者死亡率更高。
