Autoimmune Hepatitis

By Stephen Holt, MD, PhD, FACP

• What is Autoimmune Hepatitis?
• Who is at Risk?
• Causes
• Signs and Symptoms
• How is it Diagnosed?
• Treatment
• What Should I Expect During and After Treatment?
• Are There Any Alternative Treatments?
• Suggested Articles

What is Autoimmune Hepatitis?

Autoimmune hepatitis (AIH) is an uncommon liver disease which is characterized by inflammation of the liver and/or liver damage caused by an attack on the liver by a person’s own immune system. Because autoimmune hepatitis is chronic, (lasting for many years), if left untreated, it has the potential to lead to cirrhosis (scarring of the liver) and liver failure. However, when diagnosed and treated early, it can often be managed effectively with drugs by suppressing the immune system.

Who is at Risk?

About 80 percent of those affected by autoimmune hepatitis are women, usually between the ages of fifteen and forty. It also affects both much older and much younger individuals, but this occurrence is rare.

There are two types:

• Type 1 (Classic) autoimmune hepatitis. This is the most common form  and can come on suddenly and can occur at any age but affects mostly young women. This form of AIH is usually associated with other autoimmune disorders such as type 1 diabetes, Hashimoto’s thyroiditis, Graves’ disease and ulcerative colitis
• Type 2 autoimmune hepatitis. This is the less common form and mostly affects young girls.

Causes

It is not known exactly why the immune system attacks the liver causing autoimmune hepatitis. However, it is believed that this disease occurs in a genetically predisposed person triggering the body to initiate an autoimmune attack against the liver. The belief is that the disease itself is not inherited, but that the susceptibility to have the disease is due to a genetic defect, therefore, it is rarely found in more than one member of the same family. It is also thought that some diseases, toxins and drugs may trigger autoimmune hepatitis in susceptible people, especially women.

Signs and Symptoms

Signs and symptoms of can range from minor to severe and may come on suddenly or develop over time. The most common symptom is fatigue. Initially, symptoms can mimic a mild case of the flu. For some people, few, if any problems exist in the early stages of the disease, whereas others experience various signs and symptoms.

Symptoms can include:

• Loss of appetite
• An enlarged liver
• Jaundice  (yellowing of the skin and whites of the eyes)
• Abdominal discomfort
• Dark urine
• Pale stools
• Nausea
• Vomiting
• Itching
• Skin rashes
• Spider angiomas (abnormal blood vessels on the skin)
• Joint pain
• Loss of menstrual period in women

More serious complications can include ascites (fluid in the abdomen), and mental confusion.

How is it Diagnosed?

To confirm a diagnosis of autoimmune hepatitis, blood tests are performed to rule out other forms of hepatitis and other disorders with similar symptoms. Antibody tests will also pinpoint what type of autoimmune hepatitis exists. Finally, a liver biopsy is performed to confirm the diagnosis and determine the extent of damage to the liver. During the biopsy procedure, a thin needle is inserted through the skin to the liver, removing a small piece of liver tissue which is then sent off to a laboratory for analysis.

Testing is strongly suggested for individuals presenting with symptoms that are not taking drugs, not abusing alcohol and not having a family history of any metabolic liver disease or evidence of viral hepatitis.

Treatment

Some people with mild forms of the disease may not need to take medication to treat autoimmune hepatitis, but only a doctor’s careful assessment can determine whether a particular individual should undergo treatment.

For those individuals requiring treatment, the earlier the diagnosis, the better chance of slowing progress of the disease. Medications used to control the immune system (immunosuppressants) and treat autoimmune hepatitis include the following:

• Prednisone – A type of steroid/corticosteroid, prednisone is initially dispensed at a high dosage until signs and symptoms improve, at which time the dosage is lowered to the lowest dosage possible to control the disease
• Azathioprine (Imuran) – Like prednisone, azathioprine is used to suppress the immune system. Azathioprine may be used in conjunction with prednisone which allows a lower dose of prednisone to be used, thereby lowering the risk of side effects or, once the disease is under control, Azathioprine may be added later.
• Other immunosuppressants – For those individuals that don’t respond to prednisone or azathioprine, stronger immunosuppressants may be prescribed, such as cyclosporine (Sandimmune), mycophenylate mofetil (Cellcept) and tacrolimus (Prograf).
• Liver transplant – When medications don’t halt the progress of the disease, when irreversible scarring (cirrhosis) develops, or liver failure, liver transplantation is an effective procedure. The disease almost never recurs in the transplanted liver, possibly due in part to powerful drugs used to suppress the immune system to prevent transplant rejection. Transplantation has a one year survival rate of 90 percent and a five year survival rate of 70-80 percent.

What is the Outcome During/After Treatment?

In about 7 out of 10 individuals, the disease goes into remission within 3 years of starting treatment. Some people can eventually discontinue treatment although others will see symptoms return. Because autoimmune hepatitis cannot be cured, most people need to continue taking prednisone for years and sometimes for life. Unfortunately, long term use of steroids may cause serious side effects such as diabetes, high blood pressure, glaucoma, weight gain and decreased resistance to infection, therefore, other drugs may be necessary to treat the side effects.

Azathioprine can lower white blood cell counts and sometimes causes nausea and poor appetite. Rare side effects are allergic reaction, liver damage and pancreatitis, which is an inflammation of the pancreas with severe stomach pain.

Is There an Alternative Treatment?

Due to fears of instigating unknown interactions or confusing the results of any given treatment, alternative therapies are rarely permitted within the confines of Western medicine. Exceptions to this norm are most likely to be made for conditions where it has been irreversibly proven to be effective or if there are no other known medical treatments. Some people in acute liver failure who are unable to receive a liver transplant are among those for whom Western medicine has few options. Such individuals are ideal candidates for research on supplements with a track record for preserving liver health – such as N-Acetyl Cysteine.

Although several types of acute liver failure can be reversed with swift pharmaceutical intervention, many people with fulminant hepatic failure can only be saved with a liver transplant. Unfortunately, not everyone who needs a new liver can get one.

For the most common cause of acute liver failure, acetaminophen poisoning, an antidote must be administered as soon as possible to prevent liver injury. The antidote used in hospitals is N-Acetyl Cysteine (NAC). This readily available antioxidant works by indirectly replenishing glutathione – which detoxifies the toxic metabolite of acetaminophen.

According to Anne M. Larson, M.D., of the University of Washington, and colleagues, N-Acetyl Cysteine administered within 12 hours of ingestion of acetaminophen can prevent liver injury; however, many people may be unaware that they could benefit from N-Acetyl Cysteine. With a similar thought in mind, researchers from UT Southwestern Medical Center and their colleagues at 21 other institutions investigated if NAC could treat acute liver failure due to causes other than acetaminophen poisoning.

As published in the September 2009 issue of the journal Gastroenterology, the UT researchers found that acute liver failure patients in the early stages of hepatic comas who were administered NAC were nearly 2.5 times more likely to survive than those treated only with a placebo. According to Dr. William M. Lee, professor of internal medicine at UT Southwestern and lead author of this study, “NAC is safe, easy to administer, doesn’t require intensive care and can be given in community hospitals. NAC is an excellent treatment for non-acetaminophen acute liver failure if the disease is caught early.” Until this study, liver transplantation was the only treatment if the failure was from non-acetaminophen causes.

By combining Lee’s NAC results with Larson’s sentiments about its potential benefits, those with chronic liver disease could profit in a big way. If this commonly available antioxidant is good enough for preventing liver failure, shouldn’t it then be considered for this severe condition’s predecessor, liver damage? Already aware of NAC’s ability to protect the liver from damage, many healthcare practitioners advise their patients with chronic liver problems to supplement with NAC. Before waiting for liver disease to advance to liver failure, those with hepatic concerns are encouraged to investigate how N-Acetyl Cysteine could help their liver detoxify poisons – thus preventing the need for a future liver transplant.

This information is intended for educational purposes only and should not be used in any other manner. This information is not intended to substitute for informed medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified health care provider.

Suggested Articles

• Autoimmune Hepatitis
• N-Acetyl Cysteine Treats Acute Liver Failure

About the Author

Stephen Holt, MD, PhD, FACP

Stephen Holt, M.D. is a Distinguished Professor of Medicine NYCPM (Emerite) and a medical practitioner in New York State. He has published many peer-review papers in medicine and he is a best-selling author with more than twenty books in national and international distribution. He has received many awards for teaching and research. Dr. Holt is a frequent lecturer at scientific meetings and healthcare facilities throughout the world. He is a best selling author and the founder of the Holt Institute of Medicine.