A Rare Case of Type II Autoimmune Hepatitis

Abstract

Introduction: While type I autoimmune hepatitis (AIH) is widely studied in all demographics, type II AIH is far less common and classically occurs in female children. While rare in adults, it can initially present as fulminant hepatitis. Early consideration of this diagnosis is paramount in the prevention of poor outcomes. An 18-year-old female was referred for evaluation of painless jaundice. On initial presentation, she was 8 months post-partum after an uncomplicated vaginal delivery and had no other medical history. She first noted jaundice 5 days prior to presentation. Review of systems was otherwise negative. She denied history of drug, alcohol, or acetaminophen use. Her only medication was etonogestrel, a hormone-releasing implant, placed 6 months prior. Laboratory values showed a total bilirubin of 15.6, AST-1099, ALT-942, and Alk Phos-218. CT of the abdomen was significant only for calcified granulomas in the liver of unclear etiology. Initial viral and autoimmune work-up was negative, as was testing for Wilson’s and hemochromatosis. She underwent transjugular liver biopsy that revealed massive panlobular/panacinar necrosis involving 70% of the parenchyma with interface hepatitis, consistent with fulminant hepatitis. Possible etiologies included autoimmune, viral, druginduced, and Wilson’s. Further staining revealed no evidence of iron or copper. The patient underwent extensive serologic testing. Over the next several days, she had progressive declining of liver function, with worsening coagulopathy and the development of encephalopathy. She was started empirically on steroids for AIH. Given worsening mental status, she had an ICP monitor placed and was listed status 1A for transplant. Cerebral edema continued to increase and an organ offer became available. The patient underwent emergent liver transplantation. Post-operatively, she had persistently elevated ICP, and continued to require pentobarbital and hyperosmolar therapy. She developed severe hypernatremia as high as 195, necessitating termination of hyperosmolar therapy. On post-op day 5, she was taken to the OR for closure of the abdomen. That night, she rapidly declined, at which time she was found to have severe anion-gap acidosis, possibly related to sepsis and propylene glycol toxicity. She became unstable and required pressor support, and deteriorated before CVVH could be initiated. She suffered a PEA arrest and passed. Pathologic evaluation of her native liver re-demonstrated findings detailed in the initial biopsy. Anti-LKM AB was positive. This case of an 18-year-old female with type 2 AIH that was rapidly progressive underlies the importance of early recognition and empiric treatment to avoid life-threatening complications.