Idiopathic Autoimmune Chronic Active Hepatitis (IACAH)
The mean age range of the affected women is 36 to 45 years. IACAH can present in the pediatric age group. Liver and spleen enlargement are frequent while fluid in the belly and coma are infrequent. One fourth of the patients usually present with an acute episode of viral hepatitis with liver symptoms persisting of several months after the episode. Diagnosis is established by combination of clinical features, polyclonal hyperglobulinemia, raised aminotransferase levels, evidence of varying degrees of hepatocellular death and monocellular inflammatory cell infiltrate on liver biopsy.
Corticosteroids form the mainstay of medical therapy. The patients who seem to benefit the most are those with histological manifestations of liver cell death, marked biochemical abnormality and clinically apparent disease. Steroid therapy appears to improve survival but does not prevent progression to liver cirrhosis. Combination of Azathioprine with steroids does not seem to offer better therapeutic response. In general, patients who do not show remission with 4 years of steroid therapy and those with poor liver synthetic functions should be considered for liver transplantation. Early IACAH which responds to steroid therapy can achieve survival results similar to transplantation।
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