各种自身免疫性肝病的独特特征：台湾一项基于医院的22年研究。

Distinct characteristics of various autoimmune liver diseases: A 22-year hospital-based study in Taiwan.

作者信息

Chang Ming-Ling, Le Puo-Hsien, Chen Wei-Ting, Chen Tai-Di, Su Chung-Wei, Chen Cheng-Jen, Lin Cheng-Yu, Wu Chi-Huan, Kuo Chia-Jung, Sung Kei-Feng, Chien Rong-Nan

机构信息

Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, New Taipei City, Taiwan.

Department of Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan.

出版信息

J Gastroenterol Hepatol. 2024 Dec;39(12):2835-2844. doi: 10.1111/jgh.16736. Epub 2024 Sep 22.

DOI:10.1111/jgh.16736

PMID:39307997

Abstract

BACKGROUND AND AIM

The characteristics of autoimmune liver diseases (AILDs), including primary biliary cholangitis (PBC), autoimmune hepatitis (AIH), and PBC-AIH overlap syndrome (OS), have rarely been investigated and compared in Asia.

METHODS

At the Taiwan tertiary referral center, 330 PBC patients (87% treated with ursodeoxycholic acid [UDCA]), 143 AIH patients (94.4% treated with immunosuppressive therapy [IST]) and 21 PBC-AIH OS patients (85.7% treated with UDCA and IST) were enrolled.

RESULTS

Compared with AIH patients, PBC patients were older at baseline and had greater female-to-male sex ratios, alkaline phosphatase (ALP) and γ-glutamyl transferase (γ-GT) levels, and liver cirrhosis (LC), dyslipidemia, and hepatic and cardiometabolic complication rates. PBC patients had the lowest transaminase levels, whereas AIH patients had the highest transaminase levels. PBC patients had greater 22-year all-cause mortality and liver transplantation (ACMaLT) (43.5 vs 25.4%, P = 0.004), LC (75 vs 58.5%, P < 0.01), dyslipidemia (54.4 vs 45.9%, P = 0.001), and cerebrovascular accident (11.3 vs 0.8%, P = 0.019) cumulative incidences (CIs) than did AIH patients; PBC-AIH OS patients had greater systemic lupus erythematosus (28.9 vs 8.9%, P = 0.009) CI than did PBC patients. Baseline ALP (hazard ratio: 1.001), albumin (0.514), platelet count (0.997), and LC (3.438) were associated with ACMaLT; age (1.110), albumin (0.350), cirrhosis (46.219), and hepatitis C virus antibody positivity (5.068) were associated with hepatocellular carcinoma (HCC); and female sex (2.183) and body mass index (1.054) were associated with autoimmune diseases.

CONCLUSIONS

Compared with AIH patients, PBC patients had greater cardiometabolic CI, and ACMaLT CI, which was associated with cholestasis, liver functional reserve and LC. Older AILD patients with LC and females with obesity demand special caution for the development of HCC and extrahepatic autoimmune diseases, respectively.

摘要

背景与目的

自身免疫性肝病（AILD）的特征，包括原发性胆汁性胆管炎（PBC）、自身免疫性肝炎（AIH）以及PBC-AIH重叠综合征（OS），在亚洲地区鲜有研究和比较。

方法

在台湾三级转诊中心，纳入了330例PBC患者（87%接受熊去氧胆酸[UDCA]治疗）、143例AIH患者（94.4%接受免疫抑制治疗[IST]）以及21例PBC-AIH OS患者（85.7%接受UDCA和IST治疗）。

结果

与AIH患者相比，PBC患者基线时年龄更大，女性与男性的性别比更高，碱性磷酸酶（ALP）和γ-谷氨酰转移酶（γ-GT）水平更高，肝硬化（LC）、血脂异常以及肝脏和心脏代谢并发症发生率更高。PBC患者转氨酶水平最低，而AIH患者转氨酶水平最高。PBC患者22年全因死亡率和肝移植（ACMaLT）（43.5%对25.4%，P = 0.004）、LC（75%对58.5%，P < 0.01）、血脂异常（54.4%对45.9%，P = 0.001）以及脑血管意外（11.3%对0.8%，P = 0.019）的累积发病率（CI）均高于AIH患者；PBC-AIH OS患者系统性红斑狼疮的CI（28.9%对8.9%，P = 0.009）高于PBC患者。基线时ALP（风险比：1.001）、白蛋白（0.514）、血小板计数（0.997）和LC（3.438）与ACMaLT相关；年龄（1.110）、白蛋白（0.350）、肝硬化（46.2,19）和丙型肝炎病毒抗体阳性（5.068）与肝细胞癌（HCC）相关；女性（2.183）和体重指数（1.054）与自身免疫性疾病相关。