Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Autoimmune Disease

One job of the immune system is to protect the body from viruses, bacteria, and other living organisms. Usually, the immune system does not react against the body's own cells. However, sometimes it mistakenly attacks the cells it is supposed to protect. This response is called autoimmunity. Researchers speculate that certain bacteria, viruses, toxins, and drugs trigger an autoimmune response in people who are genetically susceptible to developing an autoimmune disorder.

Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Autoimmune hepatitis affects the liver.

Autoimmune hepatitis is a disease in which the body's immune system attacks liver cells. This causes the liver to become inflamed (hepatitis). Researchers think a genetic factor may predispose some people to autoimmune diseases. About 70 percent of those with autoimmune hepatitis are women, most between the ages of 15 and 40.

The disease is usually quite serious and, if not treated, gets worse over time. It's usually chronic, meaning it can last for years, and can lead to cirrhosis (scarring and hardening) of the liver and eventually liver failure.

Autoimmune hepatitis is classified as either type I or II. Type I is the most common form in North America. It occurs at any age and is more common among women than men. About half of those with type I have other autoimmune disorders, such as thyroidosis, Graves' disease, Sjögren's syndrome, autoimmune anemia, and ulcerative colitis. Type II autoimmune hepatitis is less common, typically affecting girls ages 2 to 14, although adults can have it too.

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Causes of Autoimmune Hepatitis

Autoimmune hepatitis occurs when the body's immune system, which ordinarily attacks viruses, bacteria and other pathogens, instead targets the liver. This attack on your liver can lead to chronic inflammation and serious damage to liver cells. Just why the body turns against itself is unclear, but researchers think autoimmune hepatitis could be caused by an interaction between several risk factors, such as infections, medications and a genetic predisposition.

Types of autoimmune hepatitis 

Doctors have identified two main forms of autoimmune hepatitis:
  • Type 1 (classic) autoimmune hepatitis. This is the most common type of the disease. It can occur at any age. About half the people with type 1 autoimmune hepatitis have other autoimmune disorders, such as thyroiditis, rheumatoid arthritis or ulcerative colitis.
  • Type 2 autoimmune hepatitis. Although adults can develop type 2 autoimmune hepatitis, it's most common in young girls and often occurs with other autoimmune problems.

Risk factors of Autoimmune Hepatitis

Factors that may increase your risk of autoimmune hepatitis include:

  • Being female. Although both men and women can develop autoimmune hepatitis, the disease is far more common in women.
  • Age. Type 1 autoimmune hepatitis can occur at any age. Type 2 primarily affects young girls.
  • A history of certain infections. Autoimmune hepatitis may develop after a bacterial or viral infection.
  • Use of certain medications. Certain medications, such as the antibiotic minocycline (Dynacin, Minocin, others) and the cholesterol medication atorvastatin (Lipitor), have been linked to autoimmune hepatitis.
  • Heredity. Evidence suggests that a predisposition to autoimmune hepatitis may run in families.
  • Having an autoimmune disease. People who already have an autoimmune disease may be more likely to develop autoimmune hepatitis.

Complications of Autoimmune Hepatitis

Autoimmune hepatitis may be associated with a variety of other autoimmune diseases, including:
  • Pernicious anemia. Associated with a number of autoimmune disorders, pernicious anemia occurs when a lack of vitamin B-12 interferes with your body's ability to form red blood cells.
  • Hemolytic anemia. In this type of anemia, your immune system attacks and breaks down red blood cells faster than your bone marrow can replace them.
  • Ulcerative colitis. This inflammatory bowel disease can cause severe bouts of watery or bloody diarrhea and abdominal pain.
  • Autoimmune thyroiditis (Hashimoto's thyroiditis). In this condition, the immune system attacks the thyroid gland.
  • Rheumatoid arthritis. Rheumatoid arthritis occurs when the immune system attacks the lining of your joints, leading to stiffness, pain, swelling, and sometimes deformity and disability.
  • Celiac disease. This disease causes an abnormal reaction to gluten, a protein found in many grains. Eating gluten sets off an immune response that damages the small intestine.
Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Complications of liver damage:

Autoimmune hepatitis that goes untreated can cause permanent scarring of the liver tissue (cirrhosis). Complications of cirrhosis include:

  • Increased blood pressure in the portal vein. Blood from your intestine, spleen and pancreas enters your liver through a large blood vessel called the portal vein. If scar tissue blocks normal circulation through your liver, this blood backs up, leading to increased pressure within the portal vein (portal hypertension).
  • Enlarged veins in your esophagus (esophageal varices). When circulation through the portal vein is blocked, blood may back up into other blood vessels, mainly those in your stomach and esophagus. The blood vessels are thin walled, and because they're filled with more blood than they're meant to carry, they're likely to bleed. Massive bleeding in the esophagus from these blood vessels is a life-threatening emergency that requires immediate medical care.
  • Fluid in your abdomen (ascites). Liver disease can cause large amounts of fluid to accumulate in your abdomen. Ascites can be uncomfortable and may interfere with breathing and is usually a sign of advanced cirrhosis.
  • Liver failure. This occurs when extensive damage to liver cells makes it impossible for your liver to function adequately. At this point, a liver transplant is the only option.
  • Liver cancer. People with cirrhosis have an increased risk of liver cancer.

Symptoms of Autoimmune Hepatitis

Fatigue is probably the most common symptom of autoimmune hepatitis. Other symptoms include:
  • enlarged liver 
  • jaundice
  • itching 
  • skin rashes 
  • joint pain
  • abdominal discomfort
People in advanced stages of the disease are more likely to have symptoms such as fluid in the abdomen (ascites) or mental confusion. Women may stop having menstrual periods.

Symptoms of autoimmune hepatitis range from mild to severe. Because severe viral hepatitis or hepatitis caused by a drug--for example, certain antibiotics--has the same symptoms, tests may be needed for an exact diagnosis. Your doctor should also review and rule out all your medicines before diagnosing autoimmune hepatitis.

Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Diagnosis of Autoimmune Hepatitis

Your doctor will make a diagnosis based on your symptoms, blood tests, and liver biopsy.

Blood tests. 
A routine blood test for liver enzymes can help reveal a pattern typical of hepatitis, but further tests, especially for autoantibodies, are needed to diagnose autoimmune hepatitis. Antibodies are proteins made by the immune system to fight off bacteria and viruses. In autoimmune hepatitis, the immune system makes antinuclear antibodies (ANA), antibodies to smooth muscle cells (SMA), or liver and kidney microsomes (anti-LKM). The pattern and level of these antibodies help define the type of autoimmune hepatitis (type I or type II).

Blood tests also help distinguish autoimmune hepatitis from viral hepatitis (such as hepatitis B or C) or a metabolic disease (such as Wilson's disease).

Liver biopsy. 
A tiny sample of your liver tissue, examined under a microscope, can help your doctor accurately diagnose autoimmune hepatitis and tell how serious it is. You will go to a hospital or outpatient surgical facility for this procedure.

Treatments and drugs for Autoimmune Hepatitis

Whatever type of autoimmune hepatitis you have, the goal of treatment is to slow or stop your body's immune system from attacking your liver. This may help slow the progression of the disease.
Autoimmune Hepatitis: Causes, Symptoms,Risk factors, Complications, Diagnosis, Treatment and drugs

Treatment works best when autoimmune hepatitis is diagnosed early. With proper treatment, autoimmune hepatitis can usually be controlled. In fact, recent studies show that sustained response to treatment not only stops the disease from getting worse, but it may actually reverse some of the damage.

The primary treatment is medicine to suppress (slow down) an overactive immune system.

  • Prednisone. Doctors usually recommend an initial high dose of the corticosteroid drug prednisone for people with autoimmune hepatitis. The medication is reduced to the lowest possible dose that controls the disease over a few weeks. Most people need to continue taking the prednisone for at least 18 to 24 months, and some people remain on it for life. Although you may experience remission a few years after starting treatment, the disease often returns if the drug is discontinued.

Prednisone, especially when taken long term, can cause a wide range of serious side effects, including diabetes, thinning bones (osteoporosis), broken bones (osteonecrosis), high blood pressure, cataracts, glaucoma and weight gain.

  • Azathioprine (Azasan, Imuran). Azathioprine, another immunosuppressant medication, is sometimes used along with prednisone. Using both medications may allow you to take a smaller dose of prednisone, reducing its side effects. Side effects of azathioprine may include difficulty fighting infections and nausea. Rare side effects include liver damage, pancreas inflammation (pancreatitis) and cancer.
Other immunosuppressants. If you don't respond to prednisone or azathioprine, your doctor may prescribe stronger immunosuppressants, such as mycophenolate (CellCept), cyclosporine (Neoral, Sandimmune, others) or tacrolimus (Prograf).

When medications don't halt the progress of the disease, or you develop irreversible scarring (cirrhosis) or liver failure, the remaining option is a liver transplant.

Most people will need to take prednisone, with or without azathioprine, for years. Some people take it for life. Corticosteroids may slow down the disease, but everyone is different. In about one out of every three people, treatment can eventually be stopped. It is important to carefully monitor your condition and promptly report any new symptoms to your doctor because the disease may return and be even more severe, especially during the first few months after stopping treatment.

In about 7 out of 10 people, the disease goes into remission, with a lessening of severity of symptoms, within 2 years of starting treatment. A portion of persons with a remission will see the disease return within 3 years, so treatment may be necessary on and off for years, if not for life.

Other Treatments for Autoimmune Hepatitis

People who do not respond to standard immune therapy or who have severe side effects may benefit from other immunosuppressive agents like cyclosporine or tacrolimus. People who progress to end stage liver disease (liver failure) may need a liver transplant. Transplantation is a promising alternative, with a 1-year survival rate of 90 percent and a 5-year survival rate of 70 to 80 percent.

Side Effects for both prednisone and azathioprine

Both prednisone and azathioprine have side effects. Because high doses of prednisone are needed to control autoimmune hepatitis, managing side effects is very important. However, most side effects appear only after a long period of time.

Some possible side effects of prednisone are
  • weight gain
  • anxiety and confusion
  • thinning of the bones (osteoporosis)
  • thinning of the hair and skin
  • diabetes
  • high blood pressure
  • cataracts
Some possible side effects of Azathioprine are:

Azathioprine can lower your white blood cell count and sometimes causes nausea and poor appetite. Rare side effects are allergic reaction, liver damage, and pancreatitis (inflammation of the pancreas gland with severe stomach pain).