What is autoimmune hepatitis?Autoimmune hepatitis is a disease in which your body's immune system attacks liver cells. Autoimmune hepatitis is a condition that can lead to inflammation and scarring of the liver (cirrhosis) and eventually liver failure. Autoimmune hepatitis is typically chronic, meaning it can last for years.

Doctors have identified two main forms of autoimmune hepatitis:

  • Type 1: This type of autoimmune hepatitis can occur in anyone at any age, but it typically affects young women. About half of the people with type 1 autoimmune hepatitis have other autoimmune disorders such as rheumatoid arthritis or thyroid disorders.
  • Type 2:  This type of autoimmune hepatitis is less common, typically affecting children ages 2-14, although it can affect adults too.

What causes autoimmune hepatitis?One of the jobs of the immune system is to protect the body from viruses and bacteria. But sometimes the immune system attacks things it is meant to protect and this response is called autoimmunity. Researchers think certain bacteria, toxins, drugs and genetics trigger an autoimmune response. As many as one third of patients with autoimmune hepatitis may have other immune disorders, including type 1 diabetes, thyroiditis, celiac sprue and ulcerative colitis.

What are the symptoms of autoimmune hepatitis?Fatigue is probably the most common symptom of autoimmune hepatitis. Other symptoms include:

  • An enlarged liver
  • Jaundice
  • Itching
  • Skin rashes
  • Joint pain
  • Abdominal discomfort
  • Spider angiomas, or abnormal blood vessels, on the skin
  • Nausea
  • Vomiting
  • Loss of appetite
  • Dark urine
  • Pale or gray-colored stools

How is autoimmune hepatitis diagnosed?Your doctor will make a diagnosis of autoimmune hepatitis based on symptoms, blood tests, imaging studies (CT or ultrasound) and liver biopsy. A new technology called magnetic resonance elastography may also be recommended by your doctor.

How is autoimmune hepatitis treated?The goal of treatment is to slow down an overactive immune system and slow the progress of the disease.

Steroids: Steroids (such as prednisone) control the inflammation in the liver, which prevents further scarring. The main drawback of prednisone is side effects, which are typically more prominent at higher doses. Side effects may include weight gain, acne, bone loss, elevated blood glucose levels (potentially leading to diabetes), an increased risk of infections, cataracts, high blood pressure, mood and sleep disturbance, and others. People who require long-term prednisone are monitored carefully for these side effects. To minimize the risks of side effects, the lowest possible dose of prednisone is used.

Azathioprine or 6-mercaptopurine: A second medication, such as azathioprine {Azasan®; Imuran®} or 6-mercaptopurine (Purinethol®) and, less commonly, methotrexate, cyclosporine, tacrolimus or mycophenolate mofetil may be recommended in addition to prednisone. The benefit of adding a second medication is that it may be possible to reduce or eliminate prednisone, helping to minimize the potential side effects of prednisone. However, azathioprine and 6-mercaptopurine can cause side effects, including allergic reactions, a low white blood cell count, inflammation of the pancreas, nausea, and abnormal liver blood tests. There may be a small increased risk of certain types of cancer (such as lymphoma). Blood tests to monitor for these conditions are performed regularly while taking these medications.

Treatment for autoimmune hepatitis is continued until the disease is in remission, the treatment fails, or the person develops severe side effects from treatment. Remission is defined as a lack of symptoms, near normal levels of liver blood tests, and improvement in the appearance of liver tissue (based upon a biopsy). The initial period of remission generally occurs 12 or more months after treatment begins. Approximately 65 and 80 percent of patients achieve remission by 18 months and three years, respectively.

Approximately 50 percent of patients remain in remission or have only mild disease activity for months to years after treatment is stopped. However, most patients must eventually restart treatment because the disease becomes active again (relapse). Relapse typically occurs within the first 15 to 20 months after treatment is stopped. Relapse is more likely in those who have cirrhosis on the initial liver biopsy.

When to seek medical advice:Early signs and symptoms of autoimmune hepatitis can be mild and may resemble those of the flu. See your doctor if you have, persistent fatigue, abdominal discomfort, or joint aches unrelated to exercise. You should contact your doctor immediately if you notice yellowing of your skin and the whites of your eyes or abdominal swelling.