Autoimmune Hepatitis
Autoimmune hepatitis (AIH) is a disease in which the body’s immune system attacks the liver by mistake. This immune response causes inflammation of the liver, called hepatitis, that can eventually lead to irreversible damage with scar tissue called cirrhosis or liver failure. About 70% of those with AIH are female, and those affected are believed to have a genetic factor that is triggered by an environmental agent.
The disease can be quite serious, especially if not treated. AIH is typically chronic, meaning it lasts for years, and can lead to cirrhosis—irreversible scarring and hardening—of the liver that leads to liver failure. In some cases, AIH can present acutely with severe liver injury and result in liver failure rapidly over days to weeks if not diagnosed and treated.
Two forms of AIH have been described: type 1 and type 2.
Type 1 AIH is the most common form in North America. It can occur at any age, but most often starts in adolescence or young adulthood. About half of those with type 1 have other autoimmune disorders, such as:
- Type 1 diabetes
- Proliferative glomerulonephritis, an inflammation of blood vessels in the kidneys
- Thyroiditis, an inflammation of the thyroid gland
- Grave’s disease is the leading cause of an overactive thyroid gland
- Sjögren’s syndrome, a syndrome that causes dry eyes and mouth, and autoimmune anemia
- Ulcerative colitis, an inflammation of the colon and rectum, leading to ulcers
Type 2 AIH is less common and typically affects girls aged 2 to 14, although adults can have it too.
Symptoms
Symptoms of AIH range from mild to severe, but the most common symptom is fatigue. Other symptoms include:
- Jaundice (yellowing of the eyes and skin)
- Pruritus (diffuse itching)
- Joint pain
- Abdominal discomfort
- Nausea
- Vomiting
- Loss of appetite
- Dark urine
- Pale or chalk gray-colored stools
People in advanced stages of the disease are more likely to have symptoms related to chronic liver disease, such as fluid in the abdomen (ascites) and mental confusion (encephalopathy). Women may also stop having menstrual periods.
Diagnosis
AIH is diagnosed in patients with elevated liver tests, positive antibodies, and typical findings on liver biopsy. About 20% of patients with AIH lack antibodies. It is also important to rule out other causes of liver injury that can present similarly, such as viral hepatitis or a drug-induced liver injury.
Treatments
Treatment is most effective when AIH is diagnosed early. With proper treatment, AIH can usually be controlled. In fact, studies show that sustained treatment response prevents the disease from worsening and may reverse some of the damage.
The primary treatment is medication to suppress or slow an overactive immune system.
Both types of AIH are treated with steroids (prednisone or budesonide). Budesonide can be a good option for mild to moderate inflammation because it works like prednisone in AIH and is not associated with many of prednisone’s side effects. However, budesonide is not recommended for those with cirrhosis or acute, severe AIH. Prednisone is another option and is usually dosed anywhere from 30 to 60 mg per day and tapered to 10 to 20 mg per day as the disease is controlled. The goal is to find the lowest possible dose that will control the disease.
Another medicine, azathioprine (Imuran), is also used to treat AIH. Like steroids, azathioprine suppresses the immune system, but in a different way. Treatment may begin with both azathioprine and prednisone, or azathioprine may be added later, once the disease is under control. The use of azathioprine allows for a lower dose of prednisone to minimize its potential side effects with prolonged use.
In 70-80% of people, the disease goes into remission within 3 years of starting treatment. Remission occurs when symptoms disappear, and lab tests show significant improvement in liver function. Some people can eventually stop treatment, although many will see the disease return. People who stop treatment are monitored closely and must promptly report any new symptoms to their doctor. Treatment with low doses of prednisone or azathioprine may be necessary on and off for years, if not for life.
Some people with mild forms of disease may not need to take medication. Doctors assess each patient individually to determine whether those with mild AIH should undergo treatment.
Frequently Asked Questions
The doctor will make a diagnosis based on symptoms, blood tests, and, very likely, a liver biopsy.
- Blood tests. A routine blood test for liver enzymes can help reveal a pattern typical of hepatitis, but further tests, especially for autoantibodies, are needed to diagnose autoimmune hepatitis. Antibodies are proteins made by the immune system to fight off bacteria and viruses. Autoantibodies attack the body’s cells. In AIH, the immune system produces one or more autoantibodies. The most common are Immunoglobulin G (IgG), antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA), and antibodies to liver and kidney microsomes (anti-LKM). People with type 1 have ANA, ASMA, or both, and people with type 2 have anti-LKM. Blood tests also help distinguish autoimmune hepatitis from other diseases that resemble it, such as viral hepatitis B or C or a metabolic disease such as Wilson’s disease.
- Liver biopsy. A tiny needle-biopsy of liver tissue examined under a microscope, can help doctors accurately diagnose AIH and assess its severity. This procedure is usually performed by an interventional radiologist and is done in a hospital or outpatient surgical facility.
People who do not respond to standard immune therapy or who experience severe side effects may benefit from alternative immunosuppressive agents, such as mycophenolate mofetil, cyclosporine, or tacrolimus. People who progress to end-stage liver disease—also called liver failure—or cirrhosis may need to be evaluated for a liver transplant. Transplantation has a 1-year survival rate of 90% and a 5-year survival rate of 70-80%.
Both prednisone and azathioprine have side effects. Because high doses of prednisone are often needed to control AIH, managing side effects is very important. However, most side effects appear only after a long period of time.
Some possible side effects of prednisone are:
- Weight gain
- Anxiety and confusion
- Thinning of the bones, a condition called osteoporosis
- Thinning of the hair and skin
- Diabetes
- High blood pressure
- Cataracts
- Glaucoma
Azathioprine can lower white blood cell counts and sometimes causes nausea and poor appetite. Rare side effects are allergic reaction, liver damage, and pancreatitis (acute inflammation of the pancreas, presenting with severe abdominal pain).