Autoimmune hepatitis is a disease characterized by chronic inflammation of the liver. The best way to describe this disease is to break the term down into its composite words. Autoimmune refers to the body’s own immune system attacking another part of the body. Hepatitis refers to inflammation of the liver, which can range from mild to severe. In the vast majority of patients, autoimmune hepatitis is marked by fluctuating levels of intensity, with relapses and remissions.
The exact mechanism whereby the body’s own immune system attacks the liver is not yet known. It appears that certain types of white blood cells (the type of blood cell that usually fights infection), in addition to attacking foreign substances (e.g. germs and viruses) misread liver cells as foreign substances and start attacking these cells. The type of damage that follows is known as chronic hepatitis. A number of other conditions can cause identical patterns of liver damage. These include viruses such as hepatitis B and hepatitis C, certain types of drugs, and overload of certain metals such as copper and iron in the liver. There are also much rarer causes of chronic active hepatitis.
It is absolutely not contagious. It is generally not considered an inherited disease but a tendency to autoimmune diseases may run in some families. That is, children of patients with autoimmune hepatitis may be at slightly increased risk of developing autoimmune diseases of the thyroid or liver or arthritis. The risk, however, is only slightly greater than the normal population and thus genetic counselling is not necessary.
In many patients there will be no symptoms at all. The patient will feel perfectly healthy. The condition may be detected on a routine blood test by an elevation in levels of certain enzymes that the liver makes. Other patients may experience fatigue, decreased appetite, drowsiness, or even aches or pains in the muscles or joints. Some patients may notice jaundice or yellowish discoloration of the skin and whites of the eyes as the first symptom.
Blood tests will almost always reveal elevations of ALT and AST enzymes that the liver makes. In addition, other blood tests will reveal antibodies directed against parts of different cells, such as anti-smooth muscle antibody and antinuclear factor. Your doctor, on examining you, may find abnormalities suggestive of chronic liver disease such as a large liver, jaundice, and certain signs noticeable on the skin. Finally, a liver biopsy is usually necessary to confirm the diagnosis.
A liver biopsy involves passing a small hollow-core needle into the liver after appropriate freezing with local anaesthetic. There is a very small risk of bleeding from the biopsy and some modest discomfort associated with this procedure, but it is generally well tolerated by the vast majority of patients.
Some patients with very mild or inactive disease may not need any type of active treatment. When active drug treatment is needed, corticosteroids are the treatment of first choice. Corticosteroids are completely different from anabolic steroids used to build muscle mass. Corticosteroids such as prednisone are potent anti-inflammatory drugs which will reduce inflammation in all sites, including the liver. Your doctor may choose in addition to, or in place of corticosteroids, other drugs with anti-inflammatory activity, such as azathioprine, mycophenilate mofetil or methotrexate. All these anti-inflammatory drugs have some side effects which your doctor will explain to you in detail.
Approximately 90% of patients require some form of treatment (usually azathioprine) to maintain remission after the steroids have reduced the initial inflammation. About 10% do not require any maintenance therapy and remain in remission with no symptoms and no active inflammation in the liver, for many years. Due to the variable intensity of the disease, even with maintenance treatment, patients may suffer from relapses of inflammation periodically. Such relapses are usually treated by a temporary course of steroids.
No alternative or complementary therapies have been shown to be effective in treating autoimmune hepatitis. In particular, milk thistle or its active ingredient, silymarin, has been studied, and has no beneficial effect. Standard treatments such as prednisone have been proven to delay or prevent progression to cirrhosis and thus save lives. It is ill-advised to delay or not take standard treatments in the hope that alternative remedies may work.
Yes, this can occur but is very uncommon. The drugs used to prevent rejection of the new liver by the body’s immune system are the same or similar to those used to treat autoimmune hepatitis so recurrence is usually blocked.
Research into all aspects of autoimmune hepatitis, but particularly into its causes and treatment is going on in many different laboratories in the world. The Canadian Liver Foundation is at the forefront of funding this important research. Someday we will have a cure.
For more information about autoimmune hepatitis, liver diseases or liver health, please contact us.