Autoimmune hepatitis

Other Names: Autoimmune chronic hepatitis; AIH Autoimmune hepatitis is a disease in which the body’s immune system attacks liver cells. This immune response causes inflammation of the liver, also called hepatitis. The disease can be quite serious and, if not treated, gets worse over time, leading to cirrhosis of the liver and/or liver failure.

Riskfactors

Autoimmune hepatitis likely results from a combination of autoimmunity, environmental triggers, and a genetic predisposition. It occurs more frequently in females.

Cause

The underlying cause of autoimmune hepatitis is not known. It is possible that any of several genetic or environmental factors (such as medications or viral infections) may trigger the disease.People with another autoimmune disease or a family history of autoimmune disease may be more likely to develop autoimmune hepatitis.

Symptoms of autoimmune diseases in general are caused by the body's immune system mistakenly attacking healthy tissues and cells. In the case of autoimmune hepatitis, the body's immune system attacks healthy liver tissue, ultimately damaging the liver.

Signs and symptoms

Signs and symptoms in people with autoimmune hepatitis range from mild to severe depending on the amount of liver damage present. Symptoms are generally due to scarring of liver tissue (cirrhosis). Some people have no symptoms at first and are diagnosed after being evaluated for another health problem.Some of the most common signs and symptoms in people with autoimmune hepatitis may be nonspecific and include:

• Fatigue (the most common symptom reported).
• Nausea.
• Loss of appetite.
• Diarrhea.
• Jaundice (yellowing of the skin and whites of the eyes).

Other signs and symptoms that may develop as the disease progresses include:

• Loss of brain function (hepatic encephalopathy).
• Fluid in the abdomen (ascites).
• Swelling of the legs (edema).
• Easy bruising and bleeding.
• An enlarged spleen (splenomegaly).
• Gallstones.
• Itchy skin (pruritis) or skin rashes.
• Joint pain.
• Vomiting.
• Dark urine.
• Pale or gray-colored stools.
• Absence of menstrual periods in women (amenorrhea).

Some symptoms a person experiences may be due to other underlying heath conditions or autoimmune diseases that are associated with autoimmune hepatitis.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 100% of people have these symptoms

• Increased circulating IgG level

80%-99% of people have these symptoms

• 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

30%-79% of people have these symptoms

• Abdominal pain(Pain in stomach)
• Arthralgia(Joint pain)
• Chronic fatigue(Chronic extreme exhaustion)
• Depressivity(Depression)
• Spider hemangioma

5%-29% of people have these symptoms

• Acute hepatitis(Acute liver inflammation)
• Anxiety(Excessive, persistent worry and fear)
• Arthritis(Joint inflammation)
• Ascites(Accumulation of fluid in the abdomen)
• Cirrhosis(Scar tissue replaces healthy tissue in the liver)
• Diffuse hepatic steatosis
• Gastrointestinal hemorrhage(Gastrointestinal bleeding)
• Glomerulonephritis
• Increased total bilirubin(High bili total)
• Jaundice(Yellow skin)
• Sclerosing cholangitis
• Splenomegaly(Increased spleen size)
• Thyroiditis(Thyroid gland inflammation)
• Ulcerative colitis
• Vitiligo(Blotchy loss of skin color)

1%-4% of people have these symptoms

• Fulminant hepatitis
• Hepatocellular carcinoma
• Viral hepatitis

Daignosis

The diagnosis of autoimmune hepatitis is typically made based on a person's signs and symptoms, medical history, various blood tests, and a liver biopsy. Blood tests may be able to tell autoimmune hepatitis apart from viral hepatitis, or from other health problems that cause similar symptoms. A liver biopsy can confirm the diagnosis and determine the extent of liver damage.Sometimes a person with no symptoms is suspected of having the disease after a routine health exam or when being evaluated for another health problem.

Treatment

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.

Medications that may be used initially ("induction therapy") include :

• 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.

Eating, Diet, & Nutrition If autoimmune hepatitis leads to cirrhosis, you should eat a healthy, well-balanced diet. Researchers have not found that eating, diet, and nutrition play a role in causing or preventing autoimmune hepatitis.

Prognosis

The outcome varies. Corticosteroid medicines may slow the progress of the disease. However, autoimmune hepatitis may advance to cirrhosis. This would require a liver transplant.

Possible Complications Complications may include:

• Cirrhosis
• Side effects from steroids and other medicines
• Hepatocellular carcinoma
• Liver failure

NIH genetic and rare disease info

• NIH info on Autoimmune hepatitis

Autoimmune hepatitis is a rare disease.

Autoimmune hepatitis Resources
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