Autoimmune Hepatitis Presenting with Profound Hyperferritinemia and Transaminitis

Abstract

Purpose: Case: A 79-year-old male presented with a 3-week history of fatigue, painless jaundice, dark urine, clay-colored stools, and involuntary 5-kg weight loss. His history was negative for alcohol use, cancer, known liver disease, highrisk sexual practices, blood transfusions, intravenous drug use, or ill contacts. Labs showed AST=1305U/L, ALT=667U/L, alkaline phosphatase=284U/L, total bilirubin=32.5mg/dL, direct bilirubin=25.8mg/dL, INR=1.6, and ferritin level=16,900mcg/L. Abdominal CT showed cirrhotic liver configuration and abdominal ultrasound was unremarkable. Viral and drug-induced causes for hepatitis were excluded. Liver biopsy revealed lymphoplasmacytic infiltration without evidence of hemophagocytic syndrome or iron overload, suggesting autoimmune hepatitis (AIH) (Figure 1). Further lab evaluation revealed total protein=5.7 g/dL, albumin=2.6 g/dL, gamma-globulin=1.8 g/dL, positive antinuclear antibody=3.1U and negative anti-smooth muscle antibody. Prednisone 60 mg daily was started with initial decline of AST and ALT to the 100-300 level ranges. Despite 3 weeks of treatment, his mentation continued to decline from acute liver and renal failure. Given his poor prognosis, he received palliative care until his death. Discussion: This case highlights an unusual presentation of AIH in which an elderly male presented with extreme hyperferritinemia and liver failure. Previous data suggests that the differential diagnosis for liver disease with extremely elevated ferritin levels is limited and includes hemochromatosis, Still's disease, and hemophagocytic syndrome. The marked ferritin elevation in the setting of AIH has not, to our knowledge, been previously reported. Our case demonstrates that AIH should be considered in the differential when evaluating a patient with hyperferritinemia liver disease. The clinical presentation of AIH is markedly variable and liver failure can result if treatment is delayed.Figure 1: Liver biopsy suggests AIH. Biopsy shows moderate lymphoplasmacytic infiltrate and marked bile ductular proliferation. The zone 3 areas show focal hepatocyte necrosis.