Most commonly seen in young women with prominent hyperglobulinemia. In type I IACAH elevated titers of non organ specific antinuclear antibodies. Anti smooth muscle antibodies also commonly occur in Type I IACAH. Concurrent immunogenic disorders are also noted in this group. Anti Liver-Kidney micrososmal antibodies (LKM-1) are found in patients with type -II IACAH. Type-II occurs in younger patients and has a more aggresive course with some having a fulminant course and others having very rapid progression to cirrhosis. However good response may be noted to corticosteroid therapy in this subgroup. Patients with type III IACAH are non reactive to test for ANA and anti-LKM antibodies and possess autoantibodies to a liver derived cytokeratin (type-8 and 18). However these antibodies are non specific and may be elevated in other hepatic disorders as well. The liver disease in this group of patients is believed to be due to certain viral infections leading to alterations in the hepatic cell membrane resulting in immune activation targeted against the abnormal liver cells.
Presentation and diagnosis:
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.