Department of Gastroenterology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.
Shanghai Institute of Digestive Disease, Key Laboratory of Gastroenterology and Hepatology, Chinese Ministry of Health, Shanghai Jiao Tong University, Shanghai 200001, China.
World J Gastroenterol. 2022 Aug 21;28(31):4417-4430. doi: 10.3748/wjg.v28.i31.4417.
Autoimmune liver disease (AILD) has been considered a relatively uncommon disease in China, epidemiological data for AILD in patients with cirrhosis and acute decompensation (AD) is sparse.
To investigate the prevalence, outcome and risk factors for AILD in cirrhotic patients complicated with AD in China.
We collected data from patients with cirrhosis and AD from two prospective, multicenter cohorts in hepatitis B virus endemic areas. Patients were regularly followed up at the end of 28-d, 90-d and 365-d, or until death or liver transplantation (LT). The primary outcome in this study was 90-d LT-free mortality. Acute-on-chronic liver failure (ACLF) was assessed on admission and during 28-d hospitalization, according to the diagnostic criteria of the European Association for the Study of the Liver (EASL). Risk factors for death were analyzed with logistic regression model.
In patients with cirrhosis and AD, the overall prevalence of AILD was 9.3% (242/2597). Prevalence of ACLF was significantly lower in AILD cases (14%) than those with all etiology groups with cirrhosis and AD (22.8%) ( < 0.001). Among 242 enrolled AILD patients, the prevalence rates of primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and PBC-AIH overlap syndrome (PBC/AIH) were 50.8%, 28.5% and 12.0%, respectively. In ACLF patients, the proportions of PBC, AIH and PBC/AIH were 41.2%, 29.4% and 20.6%. 28-d and 90-d mortality were 43.8% and 80.0% in AILD-related ACLF. The etiology of AILD had no significant impact on 28-d, 90-d or 365-d LT-free mortality in patients with cirrhosis and AD in both univariate and multivariate analysis. Total bilirubin (TB), hepatic encephalopathy (HE) and blood urea nitrogen (BUN) were independent risk factors for 90-d LT-free mortality in multivariate analysis. The development of ACLF during hospitalization only independently correlated to TB and international normalized ratio.
AILD was not rare in hospitalized patients with cirrhosis and AD in China, among which PBC was the most common etiology. 90-d LT-free mortality were independently associated with TB, HE and BUN.
自身免疫性肝病（AILD）在中国被认为是一种相对罕见的疾病，关于肝硬化和急性失代偿（AD）患者中 AILD 的流行病学数据很少。
调查中国肝硬化合并 AD 患者中 AILD 的患病率、结局和危险因素。
我们从乙型肝炎病毒流行地区的两个前瞻性、多中心队列中收集了肝硬化和 AD 患者的数据。患者在 28 天、90 天和 365 天结束时或直至死亡或肝移植（LT）时进行定期随访。本研究的主要结局是 90 天 LT 无死亡率。根据欧洲肝脏研究协会（EASL）的诊断标准，在入院时和住院 28 天内评估急性慢性肝衰竭（ACLF）。使用逻辑回归模型分析死亡的危险因素。
在肝硬化和 AD 患者中，AILD 的总体患病率为 9.3%（242/2597）。在 AILD 病例中，ACLF 的患病率明显低于所有病因组的肝硬化和 AD 患者（22.8%）（<0.001）。在纳入的 242 例 AILD 患者中，原发性胆汁性肝硬化（PBC）、自身免疫性肝炎（AIH）和 PBC-AIH 重叠综合征（PBC/AIH）的患病率分别为 50.8%、28.5%和 12.0%。在 ACLF 患者中，PBC、AIH 和 PBC/AIH 的比例分别为 41.2%、29.4%和 20.6%。在 AILD 相关 ACLF 患者中，28 天和 90 天的死亡率分别为 43.8%和 80.0%。在单因素和多因素分析中，病因学对肝硬化和 AD 患者的 28 天、90 天或 365 天 LT 无死亡率均无显著影响。总胆红素（TB）、肝性脑病（HE）和血尿素氮（BUN）是多因素分析中 90 天 LT 无死亡率的独立危险因素。住院期间 ACLF 的发生仅与 TB 和国际标准化比值独立相关。
在中国，肝硬化和 AD 住院患者中 AILD 并不少见，其中 PBC 是最常见的病因。90 天 LT 无死亡率与 TB、HE 和 BUN 独立相关。
