Autoimmune hepatitis (AIH) is a chronic progressive inflammatory disorder that characterize with lobular inflammation and elevation of liver transaminases. The diagnosis of AIH is based on a combination of clinical, biochemical, immunological and histological features and the exclusion of other known causes of liver disease. It means that one fourth to one third of AIH patients could have atypical presentation and they are prone to delay in diagnosis and treatment and also it means more cases progress toward advanced liver injury including liver cirrhosis and or liver failure. Based on several reports, seronegative cases of AIH have a more aggressive course. This is just one side of the coin. Traditionally we used to pertain cryptogenic liver cirrhosis to neglected fatty liver and the question is “Are they really fatty livers or something else?” Routinely, we suppose fatty liver to sedentary life and over weight because of urban sedentary life style which has begun much more earlier than silent pandemic of NAFLD and a considerable part of those who involved by NAFLD has normal BMI, known as lean NASH. Surprisingly the mortality of lean NASH is twice NAFLD. So is it pure fatty infiltration or something else? Could it be an atypical presentation of AIH or a combination and overlap of AIH & NASH? Based on my mentor comment, we can call it toxic steatohepatitis and potentially an overlap of seronegative AIH & NASH.
AIH could have an atypical presentation as seronegative AIH.
Seronegative AIH could be more aggressive.There could be diagnosis delay in challenging with seronegative AIH.
Some cases whit NAFLD, NASH and especially lean NASH could be atypical cases of AIH or at least immunogenicity could has a role in their pathogenesis and we should be aware of this possibility.