Pediatrics Center of Excellence, Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.


Autoimmune hepatitis is (AIH) is a chronic hepatitis that occurs in children and adults of all ages. It is characterized by immunologic and autoimmune features, including circulating auto antibodies and high serum globulin concentrations. It was first described in the 1950s by term of chronic active hepatitis. It has 2 types with different auto antibodies.
Diagnosis is based upon serologic and histologic findings and exclusion of other forms of chronic liver disease.
A scoring system should be used in assessment based upon:
1) Auto anti bodie titer
2) Serum IgG level 
3) Liver histology
4) Absence of viral and other causes of hepatitis.
Clear indications for treatment:
1)   rise of aminotrasferases
2)   clinical symptoms of liver disease
3)   histological features in liver biopsy
4)   Children with AIH initial treatment involve glucocorticoid with or without azathioprine. For patients with fulminant hepatitis liver transplantation, should be kept in mind.
Remission is defined by:
1)   Resolution of symptoms
2)   Normalization of serum trasaminases
3)   Normalization of serum bilirubin and gamma globuline levels.
4)   Improvement in liver histology
5)   Treatment is continued for at least 2-5 years, glucocorticoids are with drawn first, by tapering over six weeks. Azathioprine will be with drawn.