Senin, 24 Desember 2012

Autoimmune hepatitis

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Autoimmune hepatitis

Definition:
Autoimmune hepatitis

Autoimmune hepatitis is inflammation in your liver that occurs when your body's immune system attacks your liver. Although the cause of autoimmune hepatitis isn't entirely clear, some diseases, toxins and drugs may trigger autoimmune hepatitis in susceptible people, especially women.

Untreated autoimmune hepatitis can lead to scarring of the liver (cirrhosis) and eventually to liver failure. When diagnosed and treated early, however, autoimmune hepatitis often can be controlled with drugs that suppress the immune system.

A liver transplant may be an option when autoimmune hepatitis doesn't respond to drug treatments or when liver disease is advanced.


Symptoms:

Signs and symptoms of autoimmune hepatitis can range from minor to severe and may come on suddenly or develop over time. Some people have few, if any, recognized problems in the early stages of the disease, whereas others experience signs and symptoms that may include:
  • Fatigue
  • Abdominal discomfort
  • Joint pain
  • Itching (pruritus)
  • Yellowing of the skin and whites of the eyes (jaundice)
  • An enlarged liver
  • Abnormal blood vessels on the skin (spider angiomas)
  • Nausea and vomiting
  • Loss of appetite
  • Skin rashes
  • Dark-colored urine
  • In women, loss of menstruation
When to see a doctor
Make an appointment with your doctor if you have any signs or symptoms that worry you.


Causes:

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.
Complications:

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.
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.

Treatments and drugs:

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.

Medications to control your immune system (immunosuppressants)
Medications used to treat autoimmune hepatitis include:
  • 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).
Liver transplant
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.
During a liver transplant, your diseased liver is removed and replaced by a healthy liver from a donor. Liver transplants most often use livers from deceased organ donors. In some cases, a living-donor liver transplant can be used. During a living-donor liver transplant, you receive only a portion of a healthy liver from a living donor. Both livers begin regenerating new cells almost immediately.

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