Autoimmune Hepatitis (AIH)

If untreated, autoimmune hepatitis is the most aggressive liver disease and leads to cirrhosis within only a few years. Nevertheless, it can be treated very well effectively. Early and effective treatment may allow the patient to live with almost no restriction of life expectancy or and the quality of life. Hence, especially in this disease an early diagnosis and an early start of treatment are of great importance.

Clinical picture
The clinical picture of autoimmune hepatitis is very heterogeneous. Many patients are asymptomatic for a long time. In this case, the disease is only revealed during a routine check-up or examination, e. g. by elevated liver tests. However, there are also very acute cases quickly leading to liver failure and requiring immediate intensive care treatment.

Symptoms of autoimmune hepatitis

  • fatigue or exhaustion
  • arthralgia = joint pains (mostly several joints, without reddening or swelling)
  • liver skin signs (e. g. reddening of the hands = palmar erythema)
  • in some cases jaundice especially in acute cases
  • in very acute cases: a general feeling of illness, tiredness, marked jaundice, dark urine


Diagnostics
Diagnosis is based on a combination of the clinical picture, laboratory values and the result of the tissue examination (liver histology). If autoimmune hepatitis is suspected, immunosuppressive therapy with corticosteroids is initiated. Since autoimmune hepatitis responds well to immunosuppression this may confirm a suspected diagnosis.

Laboratory values:

  • elevated liver values (GOT, GPT), often very variable
  • in most cases only slightly elevated cholestatic enzymes (AP, gamma GT)
  • elevated gamma globulines (especially IgG)
  • presence of autoantibodies (ANA, SMA, SLA/LP, LKM)
  • no indication of a viral hepatitis

At least 80 percent of the patients with autoimmune hepatitis show elevated levels of auto antibodies. Especially ANA (antinuclear antibodies) and SMA (smooth muscle antigen) can be found. In 20 % of cases SLA/LP-antibodies can be detected. While ANA and SMA can also be found in other conditions, SLA/LP-antibodies are highly specific for the diagnosis of autoimmune hepatitis.

Liver histology typically shows a picture of liver inflammation, quite similar to that of a viral hepatitis. Liver diseases caused by intoxication or metabolic dysfunction are usually easy to detect and to differentiate by tissue examinations. Every fourth patient suffers already from liver cirrhosis upon diagnosis. Hence, if an autoimmune liver disease is suspected, a liver biopsy should be part of the diagnostic measures.

Treatment
Autoimmune hepatitis can be treated well with medication. If treatment is started early enough patients have a normal life expectancy. Drugs of choice at the beginning are corticosteroids. The disease responds very well to the treatment and treatment success is an important criterion for the confirming the diagnosis.
Prednisolone is a corticosteroid which has been known for a long time and which has been proven to be effective. At the beginning of treatment it is administered in a relatively high dosage and once it is seen to be effective, dosage is first reduced quickly and then more slowly. If dosage is sufficient and the dosage reduction is adequate the disease can be controlled well within the first few months in 90 % of the cases. There are only few and mostly very temporary side effects of this kind of cortisone application.
There also exist encouraging results for the treatment of AIH with the new corticosteroid budesonide. It is said to have fewer steroid side effects. Budesonide is metabolized rather quickly in the liver and hence one hopes for less unwanted effects outside the liver. A major clinical trial recently showed that budesonide is very effective in the treatment of AIH. A final evaluation of this corticosteroid is not yet possible. Further studies will have to prove whether budesonide is a good or even better alternative to prednisolone, which over decades has proven to be highly effective. Budesonide must not be given to patients who already have cirrhosis.
In order to maintain treatment success usually permanent medication with azathioprine is necessary, in every second patient combined with a low dose of steroids. If laboratory values (GOT, GPT, IgG) are normal and the tissue sample does not show any sign of activity of the disease one can try to discontinue medication after three to four years at the earliest. However, most of the patients need life-long medication.
Liver transplantation is only very rarely required in AIH. It may be necessary if the disease is diagnosed too late or if treatment is insufficient. Furthermore, liver failure due to a very acute and severe course of the disease can necessitate liver transplantation.

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