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MoonDragon's Health & Wellness Information
AUTOIMMUNE HEPATITIS
(Chronic Active Hepatitis [CAH])


For "Informational Use Only".
For more detailed information, contact your health care provider
about options that may be available for your specific situation.




HEPATITIS INTRODUCTION

Hepatitis is an inflammation of the liver. In chronic hepatitis, inflammation continues for six months or longer, with ongoing injury to liver cells. This condition may be mild, causing little damage (called chronic persistent hepatitis), or more serious, resulting in liver-cell destruction and possible cirrhosis or liver failure (called chronic active hepatitis).

Viruses are the most common cause of chronic hepatitis. Less common causes include autoimmune disease, reactions to medications and inherited metabolic disorders.

Hepatitis B and C: Two-thirds of all cases of chronic hepatitis result from infection from hepatitis B and C viruses. Both of these viruses usually begin with mild symptoms. Over time, perhaps a decade or more, both may lead to serious complications, such as cirrhosis, liver failure due to irreversible damage and scarring, and, less commonly, liver cancer. People infected with hepatitis C have the greatest risk of developing chronic hepatitis. In fact, chronic hepatitis C is nearly equal to alcoholism as a risk factor for cirrhosis.

Hepatitis D: Hepatitis D by itself won't lead to chronic hepatitis. However, in people also infected with hepatitis B, hepatitis D may increase the risk for chronic hepatitis and worsen any associated complications.

Less-common causes: Herpes viruses can cause acute hepatitis, especially in people with an already-comprised immune system. Rarely, this may lead to chronic hepatitis. Researchers believe that other not-yet-identified viruses may cause chronic hepatitis.

Although uncommon, some medications taken for long periods may also lead to chronic hepatitis. These medications include:
  • Isoniazid for tuberculosis.

  • Methyldopa for hypertension.

  • Phenytoin for seizure disorders.

Developing chronic hepatitis from medications occurs rarely, because routine blood-test monitoring helps ensure that liver enzyme changes are noticed early. Discontinuing the medication usually reverses early liver inflammation.

Some rare, inherited metabolic disorders also can lead to chronic hepatitis. The most common of these is Wilson's disease, a condition in which the body has difficulty metabolizing copper.

Autoimmune chronic hepatitis: With this form of chronic hepatitis, the immune system mistakenly destroys the body's own liver cells. What triggers autoimmune chronic hepatitis is unknown. In most cases, it's a progressive disease that leads to cirrhosis. It may appear with other autoimmune diseases, such as systemic lupus and APL syndrome. Young women have the highest rate of autoimmune chronic hepatitis, but it may affect women and men of all ages.





AUTOIMMUNE HEPATITIS DESCRIPTION

Chronic active hepatitis (CAH), or autoimmune hepatitis, is a progressive, chronic inflammatory autoimmune disease of the liver that has been identified by a number of different names besides including autoimmune chronic active hepatitis (CAH) such as idiopathic chronic active hepatitis, and lupoid hepatitis. The reason for this inflammation is not certain, but it is associated with an abnormality of the body's immune system and is often related to the production of antibodies that can be detected by blood tests. Autoimmune hepatitis was first described in 1950 as a disease of young women, associated with increased gamma globulin in the blood and chronic hepatitis on liver biopsy. The presence of anti-nuclear antibodies (ANA) and the resemblance of some symptoms to "systemic lupus erythematosus" (SLE) led to the label "lupoid hepatitis." It later became evident that this disease was not related to SLE. The disease is now called autoimmune hepatitis. It usually occurs by itself, but it can co-exist with other autoimmune diseases, such as systemic lupus or antiphospholipid (APL) syndrome.

There are other causes of CAH besides autoimmunity. These include hepatic (liver) allergic reaction to medicinal drugs, alcohol abuse, non-A, non-B virus infections, and Wilson's disease. What differentiates autoimmune hepatitis from other types of CAH is the presence of auto-antibody markers, which may vary in titer with disease activity. Such auto-antibodies are not found in the other forms of CAH, particularly CAH associated with alcohol abuse. The one exception is a type of drug-induced CAH in which auto-antibodies are present; but with the withdrawal of the offending medicine, the conditions subside.

CAH occurs in females eight times as frequently as in males, most often in the childbearing years. Often the patient is of Northern European extraction.





SYMPTOMS

The typical patient with autoimmune hepatitis is female (70%). The disease may start at any age, but is most common in adolescence or early adulthood. Blood tests identify ANA or smooth muscle antibodies (SMA) in the majority of patients (60%). More than 80% of affected individuals have increased gamma globulin in the blood. Some patients have other autoimmune disorders such as thyroiditis, ulcerative colitis, diabetes mellitus, vitiligo (patchy loss of skin pigmentation), or Sjogren's syndrome (a syndrome that causes dry eyes and dry mouth). Other liver diseases such as viral hepatitis, Wilson's disease , hemochromatosis, and alpha-1-antitrypsin deficiency should be excluded by appropriate blood tests, and the possibility of drug-induced hepatitis is ruled out by careful questioning.

Often, no symptoms appear, at least at first. People with symptoms most commonly complain of fatigue. Fatigue worsens throughout the day and may even be debilitating. Other common symptoms include:
  • Mild upper abdomen discomfort.

  • Loss of appetite.

  • Nausea.

  • Aching joints.

If chronic hepatitis becomes more severe, people may experience additional symptoms, including:
  • Jaundice.

  • Abdominal swelling.

  • Weight loss.

  • Muscle weakness.

  • Dark urine.

  • Coma.

The most common symptoms of autoimmune hepatitis are:
  • Fatigue.

  • Abdominal discomfort.

  • Aching joints.

  • Itching.

  • Jaundice.

  • Enlarged liver.

  • Spider angiomas (tumors) on the skin.

Patients may also have complications of more advanced chronic hepatitis with cirrhosis, such as ascites (abdominal fluid) or mental confusion called encephalopathy. A liver biopsy is important to confirm the diagnosis and provide a prognosis. Liver biopsy may show mild chronic active hepatitis, more advanced chronic active hepatitis with scarring (fibrosis), or a fully developed cirrhosis.





DIAGNOSIS

Because chronic hepatitis often fails to produce early symptoms, the disorder frequently is inadvertently discovered from blood tests. If the disorder is suspected, your health care provider may examine you for jaundice, tenderness in the abdomen (especially the right upper corner where the liver is located) and signs of ascites (fluid that fills the abdomen during liver failure).

Blood tests may be performed to measure:
  • Liver enzymes (released when liver cells become inflamed or damage).

  • Bile duct enzymes.

  • Bilirubin levels (Bilirubin is a pigment produced by the breakdown of red blood cells. When elevated it causes jaundice.)

If these tests show signs of liver inflammation, you will undergo tests for viral infection with Hepatitis B and C and for antibodies that signal autoimmune hepatitis. An ultrasound or CT test (also called a computed tomography scan or a CAT scan) may be performed to assess the size of the liver. A small liver and scarred appearance suggests cirrhosis.

A liver biopsy may be recommended. Examining a biopsy specimen of liver tissue under a microscope helps to determine the amount of scarring and the extent and type of liver damage. This information helps to guide treatment and to assess your chances of developing cirrhosis and liver failure. A liver biopsy also can help to rule out other disorders such as alcoholic liver injury or fatty liver.





EXPECTED DURATION

By definition, chronic hepatitis is inflammation that continues for more than six months. With mild or nonexistent symptoms, you may have chronic hepatitis for some time before it's discovered. Treatment for some types of chronic hepatitis can eliminate active infection; however, relapse can occur, because the virus can remain dormant in cells.




PREVENTION

Usually, chronic hepatitis is caused by infection with the hepatitis B or C viruses. These viruses primarily are passed through contact with blood or other bodily fluids through sharing of needles or transfusions or during sexual contact. The reason some cases of viral hepatitis progress into chronic hepatitis and others do not remains unknown. The best prevention is to protect yourself against the hepatitis B and C viruses.

Vaccinations for hepatitis B may be suggested for health-care workers and people traveling to certain countries. Infants may routinely be vaccinated for hepatitis B. Research and carefully consider the pros and cons of vaccination before considering this controversial method of disease control.

MoonDragon's Vaccination Information: Introduction & Links

MoonDragon's Vaccination Information: Mercury, Autism, Resources, & Links

Condoms should always be used during sexual contact to help prevent infection.

Causes of and measures to prevent autoimmune chronic hepatitis remain unknown.





AUTOIMMUNE HEPATITIS TREATMENT

The 10-year survival rate in untreated patients is approximately 10%.

Drug therapy with interferon alpha is the most common treatment for chronic hepatitis B and C. The drug is injected several times a week for several months (usually five to six months, but sometimes a year). Dosage and duration of therapy continue to be tested in clinical trials to improve response and lower the relapse rate.

Common side effects with interferon include:


Combination drug therapy may produce better results. However, combination therapy is usually more expensive and may produce more adverse reactions. Examples of drugs used in combination therapy include lamivudine with interferon for chronic hepatitis B, and ribavirin with interferon for chronic hepatitis C.

Corticosteroid drugs are the main treatment of autoimmune chronic hepatitis. These rugs suppress the immune system and may decrease symptoms, improve liver condition and prolong survival. The treatment of autoimmune hepatitis is immunosuppression with prednisone alone or prednisone and azathioprine (Imuran). This medical therapy has been shown to decrease symptoms, improve liver tests, and prolong survival in the majority of patients. Therapy is usually begun with prednisone 30 to 40 mg per day and then this dosage is reduced after a response is achieved. The standard dosage used in the majority of patients is prednisone 10-15 mg per day, either alone or with azathioprine 50 mg per day. Higher doses of prednisone given long-term are associated with an increase in serious side effects, including: hypertension, diabetes, peptic ulcer, bone thinning, and cataracts. Lower doses of prednisone may be used when combined with azathioprine. The goal of treatment of autoimmune hepatitis is to cure or control the disease. In two thirds to three quarters of the patients, liver tests fall to within the normal range. Long-term follow-up studies show that autoimmune hepatitis appears more often to be a controllable rather than a curable disease, because the majority of patients relapse within six months after therapy is ended. Therefore, most patients need long-term maintenance therapy. Not all patients with autoimmune hepatitis respond to prednisone treatment. Approximately 15-20% of patients with severe disease continue to deteriorate despite initiation of appropriate therapy. This is most common in patients with advanced cirrhosis on initial liver biopsy. Such patients are unlikely to respond to further medical therapy, and liver transplantation should be considered.

Supportive care is key in coping with chronic hepatitis. A well-balanced diet and good physical fitness can help you battle fatigue and improve overall health. Limit salt intake to counteract the accumulation of fluids and bodily swelling that may occur, especially if you develop cirrhosis. Also, always talk to your health care provider before taking any additional drugs, be they prescription, non-prescription or alternative medications. Your injured liver may not be able to detoxify these medications. For more information and nutritional recommendations, see related links below:

MoonDragon's ObGyn Information: Hepatitis





WHEN TO CALL YOUR HEALTH CARE PROVIDER

Because of its frequent lack of initial symptoms, chronic hepatitis often remains undetected. If you experience persistent fatigue, the most common symptom of chronic hepatitis, make an appointment to see your health care provider. If you show signs that could come from chronic hepatitis or liver failure, such as jaundice, abdominal swelling or weight loss, you should call your health care provider for an evaluation.




PROGNOSIS

If chronic hepatitis results from infection with hepatitis B or hepatitis C, treatment can eliminate active infection. However, relapse can occur, because the virus can remain dormant in cells. Ongoing monitoring is essential.

Here is the outlook for specific forms of chronic hepatitis.
  • Persistent chronic hepatitis, a milder type of hepatitis, may last years, causing few problems, disappear with time, or may lead to cirrhosis 10 or more years later.


  • Chronic active hepatitis commonly progresses to cirrhosis and liver failure, especially when left untreated.


  • Autoimmune chronic hepatitis is likely to progress to cirrhosis. Treatment, however, can help control the symptoms and improve survival.


  • Chronic hepatitis can become life threatening if cirrhosis develops. The likelihood for developing cirrhosis depends on severity of the disease as indicated by liver biopsy and the response to treatment. When the biopsy shows more severe signs of damage (as in chronic active hepatitis), treatment can be important to help decrease the risk of developing cirrhosis even when you do not have symptoms.


  • If cirrhosis develops, the risk for developing liver cancer increases. Blood testing can screen for liver cancer.





ADDITIONAL INFORMATION

American College of Gastroenterology (ACG)
4900 B South, 31st Street
Arlington, VA 22206
Phone: (703) 820-7400
Fax: (703) 931-4520
Website: http://www.acg.gi.org/

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
East Detroit, MI 48021
Phone: (810) 776-3900
Website: http://www.aarda.org/

American Liver Foundation
75 Maiden Lane
Suite 603
New York, NY 10038
Toll-Free: (800) 465-4837
Website: http://www.liverfoundation.org/

National Institute of Diabetes and Digestive and Kidney Disorders
31 Center Drive
Bethesda, MD 20892
Phone: (301) 496-3583
Fax: (301) 496-7422
Website: http://www.niddk.nih.gov/

MoonDragon's ObGyn Information: Hepatitis

MoonDragon's Health & Wellness Information: Alcoholism

MoonDragon's Health & Wellness Information: Cirrhosis

MoonDragon's Health & Wellness Information: Jaundice

MoonDragon's Health & Wellness Information: The Liver





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