Hepatitis

Description

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, Sjgren'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.