Autoimmune hepatitis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:  ;Manpreet Kaur, MD [2]

Classification

According to European Association for the Study of the Liver, there are three types of Autoimmune hepatitis discussed below:

• AIH-1:
  • Common type of AIH (accounts almost for 90% of AIH cases)
  • Presents at any age
  • Detection of ANA, SMA

or anti-SLA/LP;

• • Association with HLA DR3, DR4, and DR13; any age at onset of variable clinical and

histopathological severity; rare failure of treatment but variable relapse rates after drug withdrawal and variable need for long-term maintenance therapy • AIH-2: accounts for up to 10% of AIH cases; detection of anti-LKM1, anti-LC1 and rarely anti- LKM3; association with HLA DR3 and DR7; onset usually in childhood and young adulthood; clinical and histopathological severity commonly acute and advanced; frequent failure of treatment and frequent relapse rates after drug withdrawal; the need for long-term maintenance therapy very common • AIH-3: SLA/LP positive, otherwise very similar to AIH-1; often Ro52-antibody positive. Possibly more severe