2.1.3 Chronic Autoimmune Hepatitis (AIH)

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Presents with chronically elevated transaminases and presence of anti-smooth muscle antibodies. It may also manifest as impending liver failure.

It is a rare condition, occurring in approximately 25 to 30 patients per million. It mainly affects women (75%). It can occur at any age. The diagnosis is made by means of chronically abnormal transaminases, increased gammaglobulins, more than 20 to 30g/l with specific increase in IgG, but can also present as acute liver failure. There are different types, of which type 1 is the most common. This is characterized by the presence of anti-smooth muscle antibodies. To confirm the diagnosis, a liver biopsy is needed that shows an aggressive form of hepatitis. A favorable response to steroids is a prerequisite for correct diagnosis.

The disease can be characterized by periods of normal transaminases with flares. About 30% of patients have cirrhosis at the time of diagnosis. These patients have a poorer long-term prognosis. There is an effective treatment with steroids. The treatment is usually lifelong; the therapy should be given for at least three years. If this first-line therapy fails, a second-line therapy can be used, including cyclosporine or tacrolimus.

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