Autoimmune hepatitis : a clinical study

Abstract

This study involved the analysis of 112 patients with autoimmune hepatitis (AIH), during which process several specific issues or problems were identified. One subgroup included pregnant patients, and another patients diagnosed at an older age (>65 years). The diagnosis of AIH is not always easy, and the investigations revealed that some patients diagnosed with AIH had features of an overlap syndrome (with primary sclerosing cholangitis) or in fact never had AIH, but as retrospectively diagnosed, had toxic hepatitis masquerading as AIH. Some patients who were initially documented as clearly having chronic hepatitis C, also had autoantibodies in their serum and were (or could have been) incorrectly diagnosed as AIH. The results showed a large variability in symptoms and complaints at the time of diagnosis of AIH. The disorder can present as an acute or chronic disease. In some patients only vague symptoms are reported and in others, the diagnosis is an incidental discovery because of abnormal liver enzymes. In contrast, other patients present with acute liver failure. The diagnosis must be considered in patients with non-viral liver disease with significantly raised ANA and SMA and high serum gammaglobulin concentrations. No fixed age predisposition was found, but rather an even spread over various age groups. The male:female ratio is 1:2.5. This disorder can for the most part be well treated with a combination therapy of corticosteroids and azathioprine. Experience shows that high doses of corticosteroids can cause serious complications, particularly in older patients. A lower dose is mostly adequate, although biochemical normalisation may take longer. In approximately half of the patients, corticosteroid-therapy was discontinued, with azathioprine as the only treatment. The long-term survival of adequately treated patients is almost equal to that of the control group. In the cohort, ten patients died: three who had presented with acute liver failure, two who later developed a hepatocellular carcinoma and one who died because of cerebral lymphoma. Three older patients died of sepsis, possibly co-induced by high doses of steroids. Five patients underwent liver transplantation, of which one died of aspergillosis.