Autoimmune hepatitis is a rare disease caused by an autoimmune attack on the liver, which results in liver inflammation, dysfunction, and can lead to cirrhosis (scarring and further dysfunction), liver failure and death if not treated. About 70% of patients with autoimmune hepatitis are women between the ages of 15 and 40. If treatment does not control liver damage, a liver transplant may be necessary.
While the cause of autoimmune hepatitis is not yet fully understood, experts believe that certain bacteria, viruses, drugs, and toxins may trigger an autoimmune response in individuals who have a genetic susceptibility to autoimmune disorders.
Two types of autoimmune hepatitis:
Type I accounts for most cases in North America; can occur at any age, and is most common in women. Approximately 50% of those diagnosed with type I autoimmune hepatitis also have other immune disorders, including type 1 diabetes, thyroiditis, Graves’ disease, ulcerative colitis, Sjogren’s syndrome, or autoimmune anemia. Type II is less common, and is seen mostly in young girls, ages 2-14 in Southern Europe, although adults can be diagnosed with it as well.
Symptoms of autoimmune hepatitis include fatigue, jaundice, itching, an enlarged liver, skin rashes, joint pain, abdominal discomfort, nausea, vomiting, lack of appetite, dark urine, pale-colored stools, 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 the condition may stop having menstrual periods.
If physicians suspect autoimmune hepatitis, they will perform a physical examination, and do certain blood tests to gauge liver function, whether a virus is present or not, and to check for the presence of certain auto-antibodies which characterize autoimmune hepatitis. They will also perform a liver biopsy to determine what liver damage exists.
Hepatologists or interventional radiologists perform biopsy in most suspected cases to secure the diagnosis and stage the degree of liver damage. After giving the patient a local anesthesia, the physician inserts a needle into the liver, guided by ultrasound imaging, and removes a small tissue sample. Pathologists examine the sample to verify whether the condition is autoimmune hepatitis, and if so, if the liver has been damaged.
Early treatment of autoimmune hepatitis is vital to control disease progression, prevent, and even reverse some liver damage. Treating this condition involves suppressing the patient's overactive immune system. This is accomplished by treating (both types of) autoimmune hepatitis with the lowest dose of prednisone that is effective. A corticosteroid, prednisone, dampens the immune response and reduces chronic liver inflammation. A recent European study suggested that budesonide, a steroid with less systemic toxicity, may be equally effective. Physicians will also usually prescribe azathioprine, or Imuran, another immunosuppressant drug which works differently than prednisone and which enables lower doses of prednisone to be given.
All patients with autoimmune hepatitis are followed closely to monitor liver function. Treatment with prednisone is usually long-term, and may sometimes be discontinued; in this case, patients should be aware that symptoms may return, and that keeping in close touch with their physician is very important. Over seventy-percent of patients with this disease will experience a remission within two years of beginning treatment. Of these, some will have a relapse, requiring repeat or even indefinite treatment with immunosuppressant drugs. In such patients, the physician attempts to use the lowest dose of immune suppressive medications needed to control the disease.
Side-effects of prednisone include weight gain, anxiety, osteoporosis, diabetes, high blood pressure, cataracts, and glaucoma. Side-effects of azathioprine include low white blood cell count, nausea, and no appetite. Rarer are pancreatitis (inflammation of the pancreas and stomach pain), liver damage, and allergic reaction.
Other immunosuppressant drugs which can be used include mycophenylate mofetil, cyclosporine, or tacrolimus.