Acute hepatitis E and liver autoimmunity in a patient with acute liver failure: two separate entities?

Hepatitis E virus (HEV) infection is increasing worldwide and it is currently a public health problem in many high income countries. In most patients it is a benign and self-limited condition. However, several cases of acute hepatitis have already been described. In immunocompromised people, pregnant woman or patients with previous chronic liver disease, HEV can lead to severe cases of acute hepatitis. The differential diagnosis of hepatitis E and drug-induced or autoimmune hepatitis (AIH) can be challenging. In fact, there are some reports about hepatitis E and AIH not being two completely different entities. We report the case of a 48-year-old female without previously known liver disease who presented with acute hepatitis. She denied exceptional drugs/dietary products' ingestion. Hepatitis A/B/C virus, Cytomegalovirus, Epstein Barr or Herpes simplex 1/2 virus acute infection was excluded. Autoimmunity panel revealed positive anti-nuclear antibodies (1/320, speckled pattern) and anti-smooth muscle antibody (1/160), with complement consumption; antimitochondrial and anti-liver specific antibodies were negative. Abdominal ultrasound excluded major lesions of the liver parenchyma, biliary tract and vasculature. The patient evolved with acute liver failure encephalopathy and coagulopathy with Model for End-Stage Liver Disease (MELD) score 33 and she was submitted to an orthototic liver transplant. Meanwhile, a positive blood RNA HEV was known. The histology of the explant showed findings commonly found in AIH. The subsequent patient evolution was favourable. The final diagnosis of acute liver failure due to acute hepatitis E in a patient with findings of a previously unknown liver autoimmune disease was established.