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Autoimmune Liver Disease – Primary Biliary Cirrhosis, Chronic Active Hepatitis

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After alcohol and viruses, auto-immunity is the commonest cause of long term liver disease. This means that your body reacts against itself and the liver is damaged. Why this happens is a mystery, but illness of this type is much commoner in women than men and can be linked with other diseases such as colitis. Primary biliary cirrhosis and Chronic Active Hepatitis are the main types of auto-immune disease.

Primary Biliary Cirrhosis
In primary biliary cirrhosis the small bile ducts in the liver are inflamed and damaged. Eventually, in severe cases, they disappear and cause cirrhosis. Because bile does not flow out of the liver normally various consequences follow. Your skin itches, and this can be so bad that it keeps you awake. You might have diarrhea and get thinner because your body cannot absorb fat properly.

In primary biliary cirrhosis, certain vitamins which are absorbed with fats are also not taken up. This can cause bone disease (vitamin D), night blindness (vitamin A), and bleeding disorders (vitamin K). The skin darkens, jaundice eventually develops, and cholesterol (the fatty substance in cells) is laid down in yellow plaques in the skin. Almost everyone who has biliary cirrhosis has a particular substance called mitochondrial antibody, in the blood, and this provides one test for the disease. Even early in the disease, a liver biopsy — a sample of liver removed by a needle and examined under a microscope — will be abnormal.
Autoimmune Liver Disease

How is it treated ?
Many cases are mild and need no special treatment. For some people, replacing the vitamin deficiencies is the answer. If itching is a problem you can take antihistamine tablets like terfenadine, or cholestyramine which binds with bile acids, or have ultraviolet light treatment. If the disease is very severe you might have to consider a liver transplant.

Many different “curative” or disease-controlling treatments have been tested, but none has been so effective and safe that all doctors believe in it. There are several drugs being investigated: prednisolone, which must be used with great care because of the chance of making bone disease worse; the powerful immunosuppressive drug cyclosporin; and ursode­oxycholic acid, more familiar as a treatment for gallstones.

Chronic Active Hepatitis
Chronic active hepatitis is a different type of inflammation which damages liver cells. But it can also eventually lead to cirrhosis. Sometimes it starts out as an illness like hepatitis before it becomes persistent, so virus causes have to be ruled out. Damage to the liver caused by drinking too much alcohol can cause a similar illness.

Tests
Blood tests usually show that the blood has persistently high levels of an enzyme called transaminase. Most people who have chronic active hepatitis also have particular antibodies in the blood. You might have a liver biopsy to prove you have this disease.

How is it treated?
The usual treatment is with the drug prednisolone perhaps together with an immunosuppressive drug, azathioprine. But side effects are difficult to deal with because high doses of drugs are needed to damp down the disease.



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