“Hey Plucky, would you ever think a liver wouldn’t
know something about hepatitis?”
“Me either, but I’ve been asked about autoimmune hepatitis and
“No way Morgan!”
I really don’t know much about it. This is what I know. And, I’ll
give you some reference places to find out more”
“Morgan, what in the heck is chromo…whatever 6?”
“As I’ve said before, I’m a liver not a brain and I don’t know much about AIH. This has been a very brief overview and if you would like to learn more, I have included a list of websites that will give you a more in-depth look at autoimmune hepatitis. I also put a list together of some online support groups that might be helpful.”
AIH in General
Hepatitis is generally described as injury to the liver with inflammation of the liver cells. Autoimmune Hepatitis (AIH) occurs when your body makes antibodies against the liver tissue because it mistakes your liver as a harmful object and begins to attack it. Although you have injury to your liver, it is more of an autoimmune disease. The exact cause is unknown, AIH, like many autoimmune diseases, is thought to be because of certain bacteria, viruses, drugs, toxins and failure of the immune tolerance mechanisms in a genetically susceptible host. Genetically, the human leukocyte antigens (HLA) on chromosome 6 are the most commonly described association with AIH.
Chromosome What?
Well Plucky, chromosomes are the structures that hold genes. Genes are the individual instructions that tell your body how to develop and function-they govern physical and medical characteristics, such a hair color, blood type, and susceptibility to disease.”
“I think I’m getting it!”
“Your body is made up of individual units called cells. You have many different kinds of cells like liver cells, skin cells, blood cells, etc. In the center of most cells you have a nucleus. That is where your chromosomes are located. So, when I said AIH can be due to your genetic susceptibility, I mean there is a genetic abnormality in the chromosome that holds genes related to your immune response-chromosome 6.”
Potential Causes of AIH
“OK, back to possible causes of AIH. Viruses such as the hepatitis viruses, measles virus, cytomegalovirus and Epstein-Barr virus can also trigger autoimmune hepatitis. However, this is difficult to assess, as viral infections may occur years before the autoimmune disease manifests symptoms, and the evidence suggesting hepatitis viruses as a trigger are very weak.
Some medications may be the culprit including oxyphenisatine (a laxative withdrawn by the U.S. Food and Drug Administration), methyldopa, nitrofurantoin, minocycline, atorvastatin, interferon, and diclofenae.
Pollutants such as carbon tetrachloride (CCI4), an organic compound once used extensively as a cleaning fluid, refrigerant, and insecticide may also account for the condition.
Another environmental pollutant is trichloroethylene (TCE), an organic solvent that was widely used as a metal degreaser.
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| Henry Kunkel, 1973 |
Discovery and Study
The earliest writings describe a form of chronic liver disease prevalent among young women and characterized by an excessive increase in serum protein and gamma globulins. In 1951, Kunkel et al, termed the condition “hypergammaglobulinemic chronic hepatitis”. Since then, it has been known by various names including lupoid hepatitis, chronic active hepatitis, chronic aggressive hepatitis, plasma cell hepatitis, and autoimmune active hepatitis.
Testing and Types
Today it is simply referred to as AIH and is classified into three types based on serum antibody profiles (blood work). Abnormal values in your liver function test (LFT) will be the first place to check. Type I is the most common with about 75% of people diagnosed while Type II is linked to the other 25%. There is also Type III which is very rare in the United States.
Several tests will be ordered to check for various autoantibodies. Type I is characterized by the presence of anti-nuclear antibody (ANA) and anti-smooth muscle antibody (SMA). 25% of patients will have cirrhosis at presentation and have association with other autoimmune diseases such as celiac disease, type I diabetes, rheumatoid arthritis, ulcerative colitis, and autoimmune thyroid disease.
Type II is characterized by the presence of anti-liver Kidney microsomal (LKM) 1 and/or anti LKM3 and/or anti-liver cytosol 1 (LC1) antibodies. The test for these autoantibodies will usually be a serum protein electrophoresis (SPEP).
Type III is characterized by the presence of anti-soluble liver antigens/liver-pancreas antibodies (SLA/LP)
Type III is characterized by the presence of anti-soluble liver antigens/liver-pancreas antibodies (SLA/LP)
Diagnosis and Treatment
Once you have been diagnosed with AIH, the general treatment involves corticosteroids that suppress the immune system. Research indicates most patients achieve full remission in about 3 years but, must stay on the medications for life or they will relapse.
REFERENCES
AASLD Practice Guidelines. Diagnosis and management of Autoimmune Hepatitis.
Hindawi Publishing Company Journals. Hepatitis research and treatment.
National Digestive Diseases Information Clearinghouse. National Institute of Diabetes and Digestive and Kidney Diseases. Autoimmune Hepatitis.
World Journal of Gastroenterology. Autoimmune Hepatitis, one disease with many faces: Etiopathogenetic, clinico-laboratory and histological characteristics.
Wikipedia. Chromosome 6 (human).
ONLINE SUPPORT GROUPS
AIH Support – provide information and support to people with AIH and their friends and family.
Drugs.com AIH support group – this is a question and answer forum.
MD Junction AIH online support group – a community of patients, family members and friends dedicated to dealing with AIH, together.
I Have Autoimmune Hepatitis – an anonymous support group with information on diagnosis, treatment, symptoms, along with personal stories and experiences with AIH. “You’re not alone.”
