Aims: This case recommends that AIH must be ruled out in all patients with persistent elevations in transaminases and bilirubin regardless of other medical considerations
Methods: Autoimmune hepatitis (AIH) is an exceedingly rare disease with an incidence rate of 0.9 to 0.15 per 100,000 population.12 Due to its rarity, it may not be considered as the primary diagnosis in patients with elevated transaminases. We present a case of a 54 year old female who consulted at our hospital for jaundice and elevations in liver tests.
Results: Her clinical history dates back 2 years when she first consulted at another institution for jaundice and marked elevation of liver tests (ALT 1388U/L, TB 6.84 mg/dL, ALP 200U/L). Her jaundice allegedly spontaneously resolved but because of persistent, though mild, elevations in liver function tests and documented insulin resistance, she was managed as case of Non-alcoholic Fatty Liver Disease (NAFLD). Our patient was maintained on Vitamin E and Ursodeoxycholic Acid (UDCA) and was generally well. Liver ultrasound and Computed Tomography at that time only showed non-specific parenchymal changes of the liver and fatty infiltration. The patient sought evaluation at our institution for recurrence of jaundice two years after the initial presentation. Laboratories showed elevated aspartate aminotransferase (AST) 2092 IU/L, alanine aminotransferase (ALT) 1613 IU/L, and total bilirubin (TB) of 22.81 mg/dL. Anti-Nuclear Antibody was positive and elevated serum IgG was elevated. A diagnosis of AIH was made and Prednisone was started at 1mg/kg. Biochemical improvement was noted however the patient continued to deteriorate manifesting with severe hepatic encephalopathy and a Systemic Inflammatory like-reaction with no apparent focus of infection. Post mortem liver biopsy showed chronic hepatitis with severe activity and cirrhosis compatible with AIH.
Conclusions: Autoimmune hepatitis is a chronic disease that affects all ages and across all populations. Clinical presentation of this disease is heterogenous and may often occur undetected in most patients. Despite its varied clinical manifestations, it generally responds well to anti-inflammatory and immunosuppresive therapy. In some rare cases, patient may present with severe jaundice and deranged prothrombin levels which characterizes fulminant hepatic failure. Liver transplantation is deemed necessary for patients who present with acute liver failure.