Published online Aug 21, 2022. doi: 10.3748/wjg.v28.i31.4417
Peer-review started: March 3, 2022
First decision: April 25, 2022
Revised: May 19, 2022
Accepted: July 25, 2022
Article in press: July 25, 2022
Published online: August 21, 2022
Processing time: 166 Days and 5.7 Hours
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%) (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 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.
Core Tip: Autoimmune liver disease (AILD) has been regarded as a relatively rare disease in China. Our study reported that the overall prevalence of AILD was 9.3% hospitalized patients with cirrhosis and acute decompensation, among which primary biliary cirrhosis was the most prevalent type. In AILD patients with cirrhosis and acute decompensation, the etiology types of AILD had no significant effect on short-term mortality, total bilirubin, hepatic encephalopathy (HE) and blood urea nitrogen were independently associated with 90-d mortality in multivariate analysis. Strategies are needed to prevent presence of HE and closely monitor the changes of liver and renal function in clinical practice. These data will be a crucial complement to the public epidemiology research on AILD in Asian-Pacific regions.