Rare Immunology News
Autoimmune hepatitis is a chronic disease of unknown cause, characterized by continuing hepatocellular inflammation and necrosis, with a potential to progress to cirrhosis
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Age of Onset
Autoimmune hepatitis (AIH) is a severe liver disease that affects both children and adults. AIH arises in genetically predisposed individuals when a trigger, such as exposure to a virus, leads to a T cell-mediated autoimmune response directed against liver autoantigens. Females are 3x likely to be affected.†
5 Facts you should know
Autoimmune hepatitis may present completely asymptomatic (12–35% of the cases), with signs of chronic liver disease, or acute or even fulminant hepatic failure
Common initial symptoms include fatigue or muscle aches or signs of acute liver inflammation including fever, jaundice, and right upper quadrant abdominal pain
The disease may occur in any ethnic group and at any age, but is most often diagnosed in patients between age 40 and 50
Anomalous presentation of MHC class II receptors on the surface of liver cells, causes a cell-mediated immune response against the body's own liver, resulting in autoimmune hepatitis
Virtually all patients have increased circulating IgG levels
Interest over time
Common signs & symptoms
Increased circulating IgG level
Anti-liver cytosolic antigen type 1 antibody positivity
Antineutrophil antibody positivity
Antinuclear antibody positivity
Elevated hepatic transaminase
High liver enzymes
Liver kidney microsome type 1 antibody positivity
Smooth muscle antibody positivity
Treatment for autoimmune hepatitis should be managed by a hepatologist, which is a doctor who specializes in liver disease. Treatment aims to suppress the immune system, which is overactive in people with this disease. In many cases, especially when the disease is diagnosed early, treatment can slow the progression of the disease and may reverse some of the liver damage that has already occurred. People who have no symptoms or have a very mild form of the disease may not need treatment. A hepatologist may evaluate symptoms and use various laboratory tests to determine when a person should begin treatment.
- Corticosteroids (such as prednisone).
- Azathioprine (often in combination with corticosteroids).
- Other immune system suppressants particularly when treatment with corticosteroids and azathioprine is not effective or causes severe side effects. Examples include mycophenolate mofetil, cyclosporine, or tacrolimus.
Most people go into remission with initial treatment within two to three years. This means that their symptoms improve, and laboratory tests show that liver function is improving. In some cases, people who achieve remission can taper off medications for a period of time. However, relapses are common, and many people need long-term management ("maintenance therapy") to keep the disease under control. Maintenance therapy may involve long-term use of much lower doses of prednisone or azathioprine, which are effective in controlling the disease is most people.
Some people do not respond to treatment or cannot continue treatment due to side effects. If the disease progresses and causes severe cirrhosis and liver failure, a liver transplant may be needed. About 10-20% of people with autoimmune hepatitis eventually need a liver transplant.
Top Clinical Trials
At the time this analysis was conducted, there were no treatments in development for this disease.
Top Treatments in Research
At the time this analysis was conducted, there were no interventional clinical studies being conducted.
† Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 https://rarediseases.info.nih.gov