Autoimmune hepatitis: diagnostic and management challenges

Abstract   There are two main types of autoimmune hepatitis (AIH): AIH type 1, characterized by anti-nuclear (ANA) and/or anti-smooth muscle (SMA) antibodies; and AIH type 2, positive for anti-liver kidney microsomal antibody type 1 (LKM1). AIH type 1 affects equally adults and children, while AIH type 2 affects mainly children. There is a female preponderance for both; AIH type 1 is associated with the HLA haplotype DR3, while AIH type 2 is associated with DR7. In young patients, both types of AIH frequently present with features of acute hepatitis. The diagnosis must be suspected and the appropriate tests sought promptly, because treatment should be started as soon as possible to avoid rapid progression to cirrhosis and liver failure. Though new drugs have been used for the treatment of AIH, including ciclosporin A, the standard treatment with prednisolone, to which azathioprine may be added as a steroid sparing agent, remains the best, with excellent short and long term results. Treatment, however, has to be finely tuned, to avoid severe steroid side effects.