Autoimmune hepatitis presenting as acute liver failure: A 20-year retrospective review of North America

Autoimmune hepatitis is acommon causeof acute liver failure.Treatmentincludessteroidsfor acute liver injury and liver transplantation in those who fail to respond or develop acute liver failure.The aim of this study is to further characterize acute liver failure secondary to autoimmune hepatitis and identify variables that predict21-daytransplant-free survival. This study included adults hospitalized with acute liver failure enrolled in the Acute Liver Failure Study Group Registry between 1998 and 2019 from 32 centers within the US.The etiology of all cases was reviewed by the Adjudication Committee, and all casesidentified as autoimmune hepatitiswereincluded.Acute liver injury was defined as an INR ≥2.0 without encephalopathy and acute liver failure as INR ≥ 1.5 with encephalopathy. Laboratory and clinical data were reviewed. Variables significantly associated with21-daytransplant-free survivalwereused to develop a multivariable logistic regression model. A total of 193 cases of acute liver failure secondary to autoimmune hepatitis were identified and reviewed.There were 161 patients (83.4%) diagnosed with acute liver failure on enrollment, and 32 (16.6%) developed acute liver failure during hospitalization.At 21 days,115 (59.6%)underwentliver transplantation, 28 (14.5%) hadtransplant-free survival, and 46 (23.8%) died before liver transplantation. Higher admission values ofbilirubin, INR, and coma grade were associated with worse outcomes. A prognostic index incorporating bilirubin, INR, coma grade, and platelet count had a concordance statistic of 0.84. Acute liver failure secondary to autoimmune hepatitis isassociated with a high short-term mortality.We developed a model specifically for autoimmune hepatitis thatmay be helpful in predicting 21-daytransplant-free survival andearly identification of patients in need of expeditedliver transplantevaluation.