Predictors of Treatment Response in Autoimmune Hepatitis in Egyptian Children

Abstract

SUMMARY A utoimmune hepatitis is a chronic disease of unknown cause, characterized by continuing hepatocellular inflammation and necrosis and tendency to progress to cirrhosis. It is prevalent in children and adults with female predominance and variable clinical presentations. Our aim was to define the demographic, biochemical and histological characters of our patients with AIH and to determine predictors of treatment response. From a retrospective study where we reviewed the medical records including the history, clinical examination, investigations and treatment of all children diagnosed with AIH. We found that AIH in Egyptian children has female predominance with 80% to 20 % in males. The disease can occur at any age (infancy, childhood and adolescence) but the highest incidence in our patients occured between 2 – 10 years. The disease can be presented with a picture of acute or chronic hepatitis with equal incidence, but chronic decompensated presentation is more common than chronic compensated presentation (30% s 20%). And acute AIH should be suspected in any child with apparent acute hepatitis of no clear etiology which does not show any sign of resolving by 4–6 weeks of clinical illness. The commonest presentation in our patients was jaundice (80%) of cases, also bleeding manifestions was not uncommon (35%) of patients. There was an associated AID in 10% of patients. And the disease occurred following HAV infection in about 17% of patients. Liver biopsy assessment is essential to evaluate the nature of the disease. Liver biopsies in our patients showed dense mononuclear and plasma cell infiltration involving both the portal and peripheral areas of the liver, interface hepatitis and hepatic regeneration. Laboratory findings include elevated liver enzymes Median AST=320.5U/L, ALT=241U/L, serum bilirubin was elevated in 80% of patients with median direct bilirubin = 2.5md/dl and hypergamma globulinemia with median gamma globulin level =3.16 gm/dl. We found a highly significant statistical difference between the laboratory work up before and after treatment indicating improvement in liver function after treatment. Autoantibodies are assessed in liver diseases mainly to diagnose autoimmune liver diseases. And the most frequent positive autoantibody is ASMA 50% and 8.3% of AIH patients were seronegative. Autoantibody detection not only assists in the diagnosis but also allows differentiation of AIH in type 1 and type 2. Type 1 AIH is the commonest 80 % versus 10% type 2 AIH.immunosuppressive treatment with corticosteroids and azathioprine. The most important consideration is not to delay treatment. We have 68.4 % of our patients treated with steroids full dose followed by azathioprine, 19.3% treated with steroids and azathioprine, 7% treated with steroids only and 5.3% treated with azathioprine only. Most of our patients had early initiation of treatment before 6 months of the onset of symptoms 82.5% and there was a statistically significant relation between early initiation of treatment and response.

The mainstay of treatment for AIH is