背景:亚洲自身免疫性肝炎(AIH)的特征大多仍难以捉摸。
方法:在台湾三级治疗癌症患者中进行了一项肝活检证实的AIH患者的队列研究。
结果:从1999年到2022年,在13,766例接受肝活检的患者中,纳入150例AIH患者。男女比例为2.26。在基线,平均年龄为51.09岁,平均丙氨酸转氨酶水平为494.11U/L,和17(11.3%)有肝硬化。除一名患者外,所有患者均为1型AIH。女性年龄较大,基线肝硬化发生率高于男性。肝硬化的23年累积发病率,肝细胞癌(HCC),死亡率/肝移植,自身免疫性疾病和肝外肿瘤占64.2%,13.3%,23.4%,30.7%和21.2%,分别。1年,2年,3年,5年,10年和20年后的免疫抑制治疗复发率为60%,78.2%,81.8%,89.1%,94.5%和100%,分别。基线关联如下:碱性磷酸酶(Alk-p)水平与免疫抑制治疗后爆发[危险比(HR):1.003;95%CIHR:1.000-1.005];患有HCC的年龄(1.072;1.010-1.138)和全因癌症(1.041;1.005-1.079);肝硬化伴死亡率/肝移植(11.933;1.984-71.00A,与1.肝硬化(1.001;1.000-1.002),和自身免疫性疾病(1.001;1.000-1.002)。
结论:在一个病毒性肝炎流行的亚洲国家,AIH患者的女性对男性和基线肝硬化率低于预期,而超过60%的患者最终发展为肝硬化。高的治疗后复发率需要谨慎监测,特别是对于基线Alk-p水平高的患者。基线年龄,肝硬化状态和ANA滴度对预后至关重要.
BACKGROUND: The characteristics of autoimmune hepatitis (AIH) in Asia mostly remain elusive.
METHODS: A cohort study of liver biopsy-proven AIH patients was conducted in a tertiary care cancer of Taiwan.
RESULTS: From 1999 to 2022, of 13,766 patients who underwent liver biopsy, 150 patients with AIH were enrolled. The female-to-male ratio was 2.26. At baseline, the mean age was 51.09 years, mean alanine aminotransferase level was 494.11 U/L, and 17 (11.3%) had cirrhosis. All except one patient had AIH type 1. The females were older and had higher baseline cirrhosis rates than did the males. The 23-year cumulative incidences of cirrhosis, hepatocellular carcinoma (HCC), mortality/liver transplantation, autoimmune diseases and extrahepatic cancer were 64.2%, 13.3%, 23.4%, 30.7% and 21.2%, respectively. The 1-year, 2-year, 3-year, 5-year, 10-year and 20-year postimmunosuppressive therapy relapse rates were 60%, 78.2%, 81.8%, 89.1%, 94.5% and 100%, respectively. Baseline associations were as follows: alkaline phosphatase (Alk-p) levels with postimmunosuppressive therapy flare [hazard ratio (HR): 1.003; 95% CI HR: 1.000-1.005]; age with HCC (1.072; 1.010-1.138) and all-cause cancer (1.041;1.005-1.079); cirrhosis with mortality/liver transplantation (11.933;1.984-71.787); and antinuclear antibody (
ANA) titers with mortality/liver transplantation (1.001;1.000-1.003), cirrhosis (1.001;1.000-1.002), and autoimmune diseases (1.001; 1.000-1.002).
CONCLUSIONS: In an Asian country endemic for viral hepatitis, the female-to-male and baseline cirrhosis rates of AIH patients were lower than expected, while over 60% of the patients eventually developed cirrhosis. The high posttherapy relapse rate warrants cautious monitoring, particularly for patients with high baseline Alk-p levels. Baseline age, cirrhosis status and
ANA titers are crucial for outcomes.