S5590 Straight to Cirrhosis: An Interesting Case of Acute Severe Autoimmune Hepatitis

Introduction: Autoimmune hepatitis (AIH) is a rare, chronic immune mediated liver disease that can affect individuals of all ages, genders, and ethnicities. Current epidemiological reports do not display a peak in disease onset for any age, however AIH does show a woman predominance in both adults and children. There are 2 different types of AIH, type 1, which is more common and usually seen in adults, and type 2, which is less common and usually seen in children. We present an interesting case of a patient who had no known history of liver disease who presented with acute severe type 1 AIH. Case Description/Methods: A 72-year-old African American woman with a history significant for hypertension, hyperlipidemia, and diabetes was admitted for hypotension. Her initial labs were significant for aspartate aminotransferase of 1163, alanine aminotransferase of 769, international normalized ratio (INR) of 1.6, and a total bilirubin of 3.9. Physical exam was significant for scleral icterus and jaundice. Abdominal imaging revealed a nodular appearance of her liver suggestive of cirrhosis. She was also found to have hypergammaglobulinemia and hypocomplementemia and was positive for anti-nuclear (ANA) and anti-smooth muscle (SMA) antibodies. Liver biopsy was obtained, and she was started on prednisone for suspected AIH. Biopsy results revealed portal tracts expanded by mononuclear cells including loose aggregates of plasma cells, lobular disarray, multifocal collapse of hepatic parenchyma, and fibrosis present throughout the parenchyma. The patient showed signs of improvement on prednisone however, 2 weeks after initiation of therapy she unfortunately developed disseminated intravascular coagulation which ultimately led to her demise. Discussion: Patients with AIH can remain asymptomatic for prolonged periods of time. In type 1 AIH 28-33% of patients have cirrhosis upon initial presentation. Diagnosis is usually confirmed with biopsy displaying interface hepatitis and positive ANA/SMA antibodies, however up to 20% of patients may be seronegative. Our patient’s presentation is consistent with the diagnosis of acute severe AIH due to her having jaundice, an INR between 1.5-2.0, no encephalopathy, and no previously recognized liver disease. The mainstay of treatment for acute severe AIH is steroid monotherapy for the first 2 weeks after presentation. Despite her having no symptoms prior to her initial presentation, her biopsy results displayed strikingly advanced disease. This case highlights just how hidden AIH can remain, even in its most advanced stages.