S5736 From Flare to Fair: Hepatic Recompensation in Autoimmune Hepatitis

Introduction: Autoimmune hepatitis (AIH) is a chronic inflammatory condition that may present as an indolent chronic liver disease, an acute flare or as acute liver failure. It is defined histologically by interface hepatitis and supported by elevated immunoglobulin G (IgG) levels and the presence of autoantibodies. Liver transplant-free survival is estimated at 66% in patients with acute-severe autoimmune hepatitis treated with corticosteroids. Case Description/Methods: A 49-year-old man with obesity and prediabetes was found to have elevated aspartate aminotransferase (AST) 82 U/L, alanine aminotransferase (ALT) 84 U/L and total bilirubin (TB) 1.6 mg/dL (0.3-1.2 mg/dL) on routine labs. Serologic testing was notable for ASMA 111 U (<20 U) and IgG 2067 mg/dL (600-1640 mg/dL). A multiphase computed tomography of the abdomen revealed cirrhotic hepatic parenchyma without focal lesions. Histology from liver biopsy demonstrated interface hepatitis with dense lymphoplasmacytic infiltrates, bridging fibrosis (stage F3) and no evidence of steatosis or steatohepatitis. Based on elevated serum aminotransaminase and IgG levels, positive serologic markers, and exclusion of other etiologies of liver disease, a histological diagnosis of AIH was made and prednisone was prescribed but not started by patient. A week later, the patient presented with profound hepatic encephalopathy (HE) and was admitted to the intensive care unit for acute encephalopathy and close airway monitoring. Labs on admission were AST 474 U/L, ALT 397 U/L, TB 24.8 mg/dL, international normalized ratio (INR) 1.4 and MELD 3.0 score of 28. Acute liver failure was diagnosed and intravenous methylprednisolone therapy was initiated with a plan for slow steroid taper afterwards. Lactulose and rifaximin were started for HE. On day 4 of steroid therapy, TB was 25.3 with INR 1.5 and a dynamic SURFASA score of -3.67 suggesting steroid responsiveness. Four months after initiation of steroids, labs showed IgG 1419 mg/dL, a MELD 3.0 score of 9 and a delta MELD of 19 from admission, demonstrating biochemical improvement. Discussion: In patients who present with acute liver failure from AIH, steroid administration may provide rapid immune modulation and stabilization of hepatic function, even in patients with signs of early decompensation. Timely steroid therapy can obviate progression and need for transplantation. The trajectory of early lab values, including INR, TB, and delta MELD, and prognostic tools such as SURFASA can assist in identifying patients likely to benefit from expedited transplant evaluation.