Patients with autoimmune hepatitis without cirrhosis had a similar survival rate as the general population, whereas those with cirrhosis had increased risks for death or liver transplantation, according to recently published data.
“While some studies report that patients with [autoimmune hepatitis (AIH)] have a good prognosis and normal survival, others contradict these findings,” Floris F. van den Brand, from the Vrije Universiteit Amsterdam, and colleagues wrote. “AIH may coexist with primary biliary cholangitis (AIH-PBC) or primary sclerosing cholangitis (AIH-PSC) and recent data suggest that particularly these patients have an impaired outcome.”
To determine survival rates among patients with AIH, van den Brand and colleagues collected data on 449 patients, 29 of whom had concomitant PBC and 35 had PSC. During 10 years of follow-up, 55 patients died at a mean age of 73 years (range, 33-94 years).
Patients with AIH had an overall increased mortality risk compared with age- and sex-matched population controls. However, patients with AIH-PBC had a similar survival as the general population while those with AIH-PSC had decreased survival rates (P = .009).
Multivariate analysis showed that age (HR = 1.1; 95% CI, 1-1.1), presence of cirrhosis (HR = 2.2; 95% CI, 1.1-4.6), four or more relapses per decade (HR = 5.7; 95% CI, 1.4-23.6), and not achieving remission (HR = 3.9; 95% CI, 1.2-12.7) were predictors for death or liver transplantation.
Additionally, patients who died or underwent transplantation due to liver-related events were more likely to have cirrhosis than patients who survived (41% vs. 19%; P = .01).
The researchers established the cause of death in 58 patients. Hepatobiliary disease accounted for 26 deaths, which marked a significant increase of liver-related mortality among patients with AIH (standardized mortality ratios = 50; 95% CI, 33-76), AIH-PBC (SMR = 48.3; 95% CI, 6.8-343.2), and AIH-PSC (SMR = 278.3; 95% CI, 89.8-863) compared with the general population.
Overall survival among patients who died due to non-liver related causes did not differ from the general population, “indicating that excess mortality is driven by the underlying liver disease,” the researchers wrote.
“The findings of our study are reassuring,” van den Brand and colleagues concluded. “Patients without cirrhosis have a normal life expectancy. Only patients with cirrhosis and particularly AIH-PSC patients have an impaired survival, which can be entirely attributed to liver-related complications.” – by Talitha Bennett
Disclosure: The authors report no relevant financial disclosures.