Hepatitis is a disease that causes inflammation in the liver. Most cases of hepatitis are caused by a virus. In people with autoimmune hepatitis, immune cells mistake the liver's normal cells for harmful invaders and attack them. About 70 percent of patients with autoimmune hepatitis are women between the ages of 15 and 40.
While the causes of autoimmune hepatitis are not yet fully understood, certain bacteria, viruses, drugs, and toxins may trigger an autoimmune response in individuals who have a genetic susceptibility to autoimmune disorders.
Autoimmune hepatitis must be treated effectively to prevent the inflammation from progressing to cirrhosis (liver scarring), liver failure, or even death. At the Center for Advanced Digestive Care (CADC) at NewYork-Presbyterian/Weill Cornell Medical Center, patients with autoimmune hepatitis receive the full range of care, including the latest medical therapy and monitoring. NewYork-Presbyterian's Center for Liver Disease and Transplantation provides liver transplantation to patients with autoimmune hepatitis whose illness progresses to the point of cirrhosis or liver failure.
Types of Autoimmune Hepatitis
There are two types of autoimmune hepatitis:
- Autoimmune hepatitis type I accounts for most cases of the illness in North America. Approximately half of patients with type I autoimmune hepatitis also have other immune disorders, such as type 1 diabetes, thyroiditis, Graves' disease, ulcerative colitis, Sjogren's syndrome, or autoimmune anemia.
- Autoimmune hepatitis type II is less common in North America and is seen mostly in young girls (age 2-14) in southern Europe.
Symptoms of Autoimmune Hepatitis
Symptoms of autoimmune hepatitis may include jaundice (yellowish tint to skin and/or eyes), fatigue, nausea, vomiting, loss of appetite, an enlarged liver, skin rashes, abdominal discomfort, dark-colored urine, light-colored stools, joint pain, and spider veins on the skin. Patients with advanced disease are more likely to have ascites (fluid in the abdomen) or encephalopathy (mental confusion), which result from chronic liver dysfunction. Women with autoimmune hepatitis may stop having menstrual periods.
Autoimmune Hepatitis Diagnosis
To diagnose autoimmune hepatitis, CADC physicians perform a physical examination and order blood tests to evaluate liver function, determine whether a virus is present, and check for the presence of certain auto-antibodies associated with the illness. They will also perform a liver biopsy (removal and examination of a sample of liver tissue) to check for liver damage.
Treatment of Autoimmune Hepatitis
Early treatment of autoimmune hepatitis is important for controlling progression of the disease and to prevent and even reverse some liver damage. As with other autoimmune diseases, treatment involves suppressing the patient's immune system.
Prednisone is the drug most commonly used to treat autoimmune hepatitis. It is a steroid that controls inflammation, dampens the immune response, and may need to be taken long-term. Patients will also usually receive azathioprine (Imuran), another immunosuppressant drug, which works differently than prednisone and which enables lower doses of prednisone to be given. Other immunosuppressant drugs that some patients take include mycophenylate mofetil, cyclosporine, or tacrolimus.
Regular visits to a physician are important for monitoring liver function in people with autoimmune hepatitis. More than 70 percent of patients experience a remission within two years of starting treatment; some will need additional or even indefinite therapy with immunosuppressant drugs. Physicians attempt to use the lowest dose of immune-suppressive medications needed to control the disease.
If treatment of autoimmune hepatitis is not effective for controlling liver damage, a liver transplant may be necessary. Liver transplantation is available through NewYork-Presbyterian Hospital's Center for Liver Disease and Transplantation.