Department of Internal Medicine, Seoul National University Bundang Hospital, College of Medicine, Seoul National University, Korea.
Clin Mol Hepatol. 2018 Mar;24(1):10-19. doi: 10.3350/cmh.2017.0066. Epub 2018 Jan 5.
Autoimmune liver diseases including autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are rare diseases. The aim of this review is to examine the epidemiology and clinical characteristics of AIH and PBC in South Korea. There were 4,085 patients registered as AIH in the Rare Intractable Disease Registry of Korea between 2009-2013, with a median age of 56 years and female-to male ratio of 6.4. The age-adjusted incidence and prevalence of AIH were 1.07/100,000/year and 4.82/100,000 persons, respectively. Among the patients, 1.1% underwent liver transplantation, and case fatality was 2.18%. Liver cirrhosis at diagnosis was accompanied in 23%; liver biopsy was performed in 75.2%, and prednisolone therapy or prednisolone and azathioprine combination therapy was done in 73% with a remission rate of 86%. There were 2,824 patients with PBC (≥20 years) registered in Korea between 2009-2013 with a median age of 57 years and female-to male ratio of 6.2. The age-adjusted incidence and prevalence of PBC were 0.86/100,000/year and 4.75/100,000 persons, respectively. Among the patients, 2.5% underwent liver transplantation, and case fatality was 2.2% with a 5-year transplantation-free survival of 95.4%. Ursodeoxycholic acid (UDCA) was prescribed in 90% of the patients with a UDCA inadequate response rate of 30%. In conclusion, AIH and PBC are rare but mostly treatable diseases if diagnosed in the early stages. However, scarce data, low awareness, delayed diagnosis and non-availability of 2nd line therapeutics are important issues to be solved. Therefore, governmental support for research and drug development and nationwide cooperative studies are warranted.
自身免疫性肝病包括自身免疫性肝炎（AIH）和原发性胆汁性胆管炎（PBC），是罕见疾病。本综述旨在探讨韩国 AIH 和 PBC 的流行病学和临床特征。在 2009-2013 年期间，韩国罕见疑难疾病登记处共登记了 4085 例 AIH 患者，中位年龄为 56 岁，男女比例为 6.4。AIH 的年龄调整发病率和患病率分别为 1.07/100,000/年和 4.82/100,000 人。其中，1.1%的患者接受了肝移植，病死率为 2.18%。诊断时伴有肝硬化的患者占 23%；75.2%的患者进行了肝活检，73%的患者接受了泼尼松龙治疗或泼尼松龙联合硫唑嘌呤治疗，缓解率为 86%。在 2009-2013 年期间，韩国共登记了 2824 例（≥20 岁）PBC 患者，中位年龄为 57 岁，男女比例为 6.2。PBC 的年龄调整发病率和患病率分别为 0.86/100,000/年和 4.75/100,000 人。其中，2.5%的患者接受了肝移植，病死率为 2.2%，5 年无移植生存率为 95.4%。90%的患者接受了熊去氧胆酸（UDCA）治疗，UDCA 应答不足率为 30%。总之，如果在早期诊断，AIH 和 PBC 是可治疗的罕见疾病。然而，数据稀缺、认识不足、诊断延迟和缺乏二线治疗药物是亟待解决的重要问题。因此，需要政府支持开展研究和药物开发，并进行全国性的合作研究。
