Autoimmune Hepatitis

Autoimmune chronic hepatitis typically occurs in women between the ages of 20
and 40 who have other autoimmune diseases, including systemic lupus
erythematosus, rheumatoid arthritis, Sjögren's syndrome, inflammatory bowel
disease, glomerulonephritis, and hemolytic anemia. Some research indicates that
the postmenopausal period may be another peak in incidence of AIH among women.
About 30% of patients are men, however, and in both genders there is often no
relationship to another autoimmune disease. In general, no major risk factors
have been discovered for this condition.
Symptoms of Autoimmune Hepatitis
About 85% of people with chronic active autoimmune hepatitis do not have severe
symptoms at all. When symptoms occur, they range from minimal to severe, and
include fatigue, jaundice, fever, and weight loss. The liver and spleen are
often enlarged. In addition, patients with this condition may experience skin
disorders, including palmar erythema (red palms) and spider angioma (a blood-red
spot, the size of a pinhead, from which tiny blood vessels radiate like spider
legs). Itching is not common, however. The abdomen or legs may be swollen due to
the accumulation of fluid.
Tests for Autoimmune Chronic Hepatitis
If a patient experiences symptoms of chronic active hepatitis for six months or
more and a virus cannot be identified, then autoimmune hepatitis is usually
suspected. There are other autoimmune liver diseases, however, that can confuse
a diagnosis. To help confirm this condition, test results may show high levels
of immune factors called serum globulins or certain antibodies to liver
proteins. In some cases, a successful trial of steroid drugs may be the only way
to diagnose autoimmune hepatitis.
Outlook for Autoimmune Hepatitis
Autoimmune hepatitis is usually benign and causes little trouble, although there
is a very small risk that it can evolve into the active form. One study reported
a 10-year survival rate of 95%, which was similar to the same age group in the
general population. However, it the condition evolves into the chronic active
form, five-year survival rate may be only 50% if the disease is not treated.
(The survival rate can be higher in people with milder symptoms and less liver
damage.)

Although very uncommon, severe autoimmune hepatitis can be life-threatening and
require intensive therapy, including possibly liver transplantation. The risk
for liver failure and bleeding in the stomach and esophagus is highest in the
early years after disease onset. This risk diminishes over time but is replaced
by an increase in liver cancer rates and bleeding in the stomach and intestines.
The risk for liver cancer is not as high, however, as with chronic viral
hepatitis.
Treatments for Autoimmune Hepatitis
Patients with autoimmune hepatitis who have mild symptoms and slight
inflammation of the liver do not require any treatment except to alleviate
symptoms. They should be monitored, however, for any signs of disease
progression. Because of effective treatment options and in spite of a high rate
of relapse, long-term survival rates in patients with autoimmune hepatitis are
excellent. Drugs that block factors in the immune system and help reduce
inflammation and symptoms of autoimmune hepatitis are most often used.

Corticosteroids. Corticosteroids, prednisone and prednisolone, are the standard
agents used for autoimmune hepatitis. They produce remission of symptoms in
about 80% of patients with autoimmune hepatitis. For most patients, steroids
also reduce symptoms within three months, improve liver function within six
months, and restore liver health within two years. Between 10% and 20% of
patients continue to deteriorate despite steroid treatment, although higher
doses may help some of these people. (Steroids are generally not useful for
chronic hepatitis B or C, and, in fact, suppressing the immune system in these
patients can encourage the viruses to replicate more quickly.)

Treatment usually needs to continue for about two years before the disease is in
complete remission. Usually, steroids are stopped when disease symptoms have
disappeared, when blood tests show that aminotransferase levels are less than
two times normal, and liver biopsies reveal no active cell damage. Steroid
medications must be withdrawn very slowly. Patients who are very elderly or who
have advanced (decompensated) cirrhosis are not good candidates for this
treatment.

Unfortunately, remission rarely lasts more than three years. About half of
patients relapse within six months, and only about 20% of patients achieve
remission (are disease-free) for more than five years. Re-administering
prednisone therapy after relapse achieves another remission in 80% of patients.

Side effects can be very distressing and sometimes serious; they include weight
gain, skin problems, moon-shaped face, high blood pressure, diabetes, cataracts,
mental disturbances, infections, and osteoporosis.

Investigative Agents. In severe cases, drugs that block the immune system may be
used:

Azathioprine (Imuran) is often prescribed along with steroids to help reduce
severe side effects caused by using steroids alone. Azathioprine also suppresses
the immune system and helps prevent relapse, but the drug will not induce
remission by itself. In one promising study, patients who continued to use
azathioprine after prednisolone was withdrawn had no relapses for at least a
year. Unfortunately, long-term use of azathioprine may increase the risk for
cancer, although studies indicate that this risk is very low.
Cyclosporine A (Neoral) is another immunosuppressant and may prove to be a
safe and effective alternative to corticosteroids.

Some important research is targeting agents that inhibit RNA--the genetic
molecules that serve as messengers for regulating cellular processes. In a 2003
animal study, an agent that targeted RNA specifically affecting cell receptors
involved in liver injury protected against autoimmune hepatitis in mice.

Liver Transplantation and Autoimmune Hepatitis. If all therapies fail and the
disease becomes life threatening, liver transplantation may be performed. Liver
transplantation is problematic, however. In one study, half of patients who
received a transplant required re-transplantation within a year. Autoimmune
hepatitis recurred in 25% of patients studied. (According to one 2000 study,
transplantation in these patients may improve accompanying autoimmune disorders
in half of patients who experienced it.) Children who develop autoimmune
hepatitis after liver transplantation may respond to corticosteroid and
azathioprine therapy.


Resources

www.cdc.gov -- Centers for Disease Control and Prevention (888-443-7232)
www.hepfi.org -- Hepatitis Foundation International (800-891-0707)
www.liverfoundation.org -- American Liver Foundation (800-465-4837)
www.aasld.org -- American Association for the Study of Liver Diseases
www.gastro.org -- American Gastrointestinal Association
http://cpmcnet.columbia.edu/dept/gi/disliv.aspl -- Information in liver
diseases
www.niddk.nih.gov -- National Institute of Diabetes and Digestive and Kidney
Diseases
www.immunize.org -- Immunization Action Coalition
www.hepnet.com -- The Hepatitis Information Network
www.hepatitis-central.com -- Hepatitis Central
www.unos.org -- United Network for Organ Sharing
www.medhelp.org/perl6/Hepatitis -- Patient-to-patient support forum
http://frontline-hepatitis-awareness.com Advocacy and patient outreach




Sandra Tara Balduf (Ane)

Frontline Hepatitis Awareness

Support for patients and educational materials

http://frontline-hepatitis-awareness.com

1-866-Hep-GoGo 866-437-4646




[Non-text portions of this message have been removed]