Nationwide survey of patients with acute liver failure and late-onset hepatic failure in Japan seen between 2016 and 2021

AimA nationwide survey was conducted to clarify the recent status of patients with acute liver failure (ALF) and late-onset fulminant hepatitis (LOHF) in Japan.MethodsTwo-step surveys were performed annually targeting 782 hospitals, focusing on patients meeting the Japanese diagnostic criteria.ResultsA total of 1404 patients seen between 2016 and 2021 were enrolled, including 1373 patients with ALF (824 non-comatose, 320 acute type, and 229 subacute type) and 31 patients with LOHF. Of these, 1117 patients (79.6%) had hepatitis, and 287 patients (20.4%) did not have hepatitis. Compared to patients seen from 2010 to 2015, those from 2016 to 2021 showed a decrease in the proportion of viral cases across all types compared to those up to 2009, whereas the proportion of drug-induced, autoimmune, and indeterminate cases increased. Among the patients, 32 had HBV reactivation due to immunosuppressive and/or antineoplastic therapies (17 HBsAg positive and 15 HBsAg negative). The frequency of complications and various treatment methods did not show significant changes compared to previous surveys. Excluding non-comatose cases, the survival rate with medical treatment for patients with and without hepatitis remained low. Liver transplantation was performed in 144 patients with hepatitis (12.9%) and in 19 patients without hepatitis (6.6%). Multivariate analysis identified disease types, patient age, etiology, liver atrophy, and complications as factors associated with the outcome.ConclusionAlthough the clinical features and etiologies of patients with ALF and LOHF have evolved, patient outcomes have not improved in recent years.