摘要
BACKGROUND 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.AIM To investigate the prevalence,outcome and risk factors for AILD in cirrhotic patients complicated with AD in China.METHODS 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 LTfree 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.RESULTS 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%)(P<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 LTfree 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.CONCLUSION 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
基金
Supported by Shanghai Hospital Development Commission,No.SHDC2020CR1037B
the National Key R&D Program of China,No.2017YFC0908100
the National Science and Technology Major Project,No.2018ZX10302206,2018ZX10723203 and 2017ZX10202202
Shanghai Municipal Education Commission-Guofeng Clinical Medicine Grant,No.20152213
the National Natural Science Foundation of China,No.82170629,81930061,81900579,81970550,82070613,82070650,and 81972265
Chongqing Natural Science Foundation,No.CSTC2019jcyj-zdxmX0004
Beijing Municipal Science&Technology Commission,No.Z191100006619033
Local Innovative and Research Teams Project of Guangdong Pearl River Talents Program,No.2017BT01S131
the Foundation for Innovative Research Groups of Hubei Provincial Natural Science Foundation,No.2018CFA031
Guangdong Basic and Applied Basic Research Foundation,No.2020A1515010052.
