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Publication Information
Affiliation
Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.; Medical University of South Carolina, Charleston, South Carolina, USA.; The University of Texas Southwestern Medical Center, Texas, USA.; Virginia Commonwealth University Medical Center, Richmond, Virginia, USA.; Mayo Clinic Arizona, Phoenix, Arizona, USA.; University of California San Francisco Medical Center, San Francisco, California, USA.; Medical University of South Carolina, Charleston, South Carolina, USA.; The University of Texas Southwestern Medical Center, Texas, USA.; University of Washington Medical Center, Seattle, Washington, USA.; Baylor St. Luke's Medical Center, Houston, Texas, USA.; Medical University of South Carolina, Charleston, South Carolina, USA.; The University of Texas Southwestern Medical Center, Texas, USA.; Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Authors
Enke Thomas, Livingston Sherry, Rule Jody, Stravitz Todd, Rakela Jorge, Bass Nathan, Reuben Adrian, Tujios Shannan, Larson Anne, Sussman Norman, Durkalski Valerie, Lee William, Ganger Daniel
Studies
Abstract
Autoimmune hepatitis is a common cause of acute liver failure. Treatment includes steroids for 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 predict 21-day transplant-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 cases identified as autoimmune hepatitis were included. 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 with 21-day transplant-free survival were used 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%) underwent liver transplantation, 28 (14.5%) had transplant-free survival, and 46 (23.8%) died before liver transplantation. Higher admission values of bilirubin, 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 is associated with a high short-term mortality. We developed a model specifically for autoimmune hepatitis that may be helpful in predicting 21-day transplant-free survival and early identification of patients in need of expedited liver transplant evaluation.