Patient education: Autoimmune hepatitis (Beyond the Basics)

AUTOIMMUNE HEPATITIS OVERVIEW — The liver is one of the largest and most important organs in the body. It functions to cleanse toxins from the blood, break down medications, and aids in digestion and blood clotting.

Hepatitis is a general term that means inflammation of the liver. There are many forms and causes of hepatitis (such as viruses and certain drugs), including autoimmune hepatitis (AIH). In AIH, the body's immune system attacks the cells of the liver, which causes the liver to become inflamed. AIH may present at any time, at any age, and in people of any race or sex, although it is four times more common in females than males.

Topics that discuss other types of hepatitis are available separately. (See "Patient education: Hepatitis A (Beyond the Basics)" and "Patient education: Hepatitis B (Beyond the Basics)" and "Patient education: Hepatitis C (Beyond the Basics)".)

AUTOIMMUNE HEPATITIS CAUSES — It is not clear why AIH develops. Researchers suspect that some people inherit a genetic disposition that could make them more likely to develop it. Sometimes drugs or infections trigger the development of the disease.

TYPES OF AUTOIMMUNE HEPATITIS — There are two major forms of AIH: type 1 and type 2.

●Type 1 AIH can affect people of any age or sex

●Type 2 AIH primarily affects girls and young females and is less common

There are also rare forms of AIH (called variants) that have features of both AIH and other liver diseases (primary sclerosing cholangitis or primary biliary cholangitis). This occurs in approximately 15 percent of people with AIH. These variant syndromes can occur along with AIH at time of diagnosis or may develop later.

AUTOIMMUNE HEPATITIS SYMPTOMS — Many people with autoimmune hepatitis (AIH) have no symptoms. The disorder is often first detected by abnormal laboratory tests performed for an unrelated reason (such as for a life insurance examination).

When symptoms are present, the most common symptom is fatigue. Some people also have additional symptoms, including jaundice (yellowing of the skin or eyes), itching, skin rashes, joint pain, abdominal discomfort, nausea, vomiting, loss of appetite, dark urine, and pale or gray-colored stools. In its most advanced form, AIH can progress to cirrhosis (severe scarring of the liver). (See "Patient education: Cirrhosis (Beyond the Basics)".)

Rarely, a person may not know they have AIH until they develop severe liver damage and acute liver failure, which may require urgent liver transplantation.

AUTOIMMUNE HEPATITIS DIAGNOSIS — AIH is diagnosed with a combination of blood tests (to exclude viruses and other causes of liver disease) and a liver biopsy. During a liver biopsy, a small sample of liver tissue is removed for examination under a microscope. The biopsy can help to confirm the diagnosis and determine its severity while excluding other causes of liver disease. (See "Patient education: Liver biopsy (Beyond the Basics)".)

AUTOIMMUNE HEPATITIS TREATMENT — Not everyone with autoimmune hepatitis (AIH) needs treatment immediately. The decision to treat is based on the severity of symptoms, the severity of the disease (based upon results of blood tests and the liver biopsy), and the potential side effects of treatment. The guidelines for treatment can be found online at the American Association for the Study of Liver Diseases (https://www.aasld.org).

Medications — AIH is usually treated first with a glucocorticoid (steroid medication) such as prednisone; budesonide may be used in people without severe liver scarring.

●Glucocorticoids – Glucocorticoids such as prednisone control the inflammation in the liver, thereby preventing further scarring. The main drawback of prednisone is side effects, which can include weight gain, acne, bone loss, elevated blood glucose levels (potentially leading to diabetes), an increased risk of infections, cataracts, high blood pressure, and mood and sleep disturbance, among 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.

Budesonide is a glucocorticoid that has more liver-specific effects and is associated with fewer side effects. It is an alternative to prednisone in selected situations. It should only be used in people who have no evidence of cirrhosis.

●Azathioprine or 6-mercaptopurine – A second medication, such as azathioprine (brand names: Azasan, Imuran) or 6-mercaptopurine (brand names: Purixan, Purinethol) and, less commonly, methotrexate 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.

Azathioprine and 6-mercaptopurine can also cause side effects, including allergic reactions, a low white blood cell count, inflammation of the pancreas, nausea, and abnormal liver blood tests (which can sometimes cause confusion as to whether the abnormal results are from the AIH or the drugs used to treat it). 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.

Mycophenolate has several potential risks, including an increased risk of developing infections or cancers (www.nlm.nih.gov/medlineplus/druginfo/meds/a601081.html). Mycophenolate can cause birth defects and should not be taken during pregnancy. Anyone who uses mycophenolate and could potentially get pregnant must use two effective methods of birth control (eg, condoms and the birth control pill).

For people with AIH who do not respond to initial treatment, other medications that may be used include an anti-rejection medication such as tacrolimus or cyclosporine. Rarely, infliximab or rituximab may be used. Liver transplantation may be needed for people with very severe disease or in people with cirrhosis who have complications.

For people taking a glucocorticoid such as prednisone, bone mineral density is monitored to screen for osteoporosis.

Duration of treatment — As a general rule, treatment 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, normal or 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. The majority of people achieve remission by 18 months to three years of treatment.

Approximately 50 percent of people remain in remission or have only mild disease activity for months to years after treatment is stopped. However, most people (75 to 80 percent) must eventually restart treatment because the disease becomes active again (relapse). Relapse typically occurs within the first 6 to 12 months after treatment is stopped. Relapse is more likely in those who have cirrhosis on the initial liver biopsy.

If medications are not used — Close follow-up is recommended for people who are not initially treated with medications. Follow-up generally includes a physical examination and blood tests every few months.

Self care — Taking medication and seeing a health care provider on a regular basis can help to ensure that the liver remains as healthy as possible.

Diet — No specific diet has been shown to improve the outcome in people with AIH. The best advice is to eat a normal, healthy and balanced diet and to avoid becoming obese; obesity can increase the risk of fatty liver disease and may complicate AIH.

Alcohol — Alcohol should be avoided since it can cause fatty liver and other liver damage. All types of alcoholic beverages can be harmful to the liver, including beer, wine, and liquor. People with liver disease may worsen with even small amounts of alcohol.

Exercise — Exercise is good for overall health and is encouraged, but it has no specific benefit for people with AIH.

Prescription and nonprescription drugs — Many drugs are broken down by the liver. Thus, it is always best to check with a health care provider or pharmacist before starting a new prescription. Unless the liver is already scarred, most drugs are safe. Some people with active liver disease will be advised to take a smaller dose of medication.

An important exception is acetaminophen (sample brand name: Tylenol), commonly used for headaches, other aches and pains, and fever. In people with any type of liver disease, the maximum recommended dose of acetaminophen is no more than 2000 mg (in divided doses) per 24 hours. Thus, it is reasonable to take 500 mg every four to six hours, although this should not be repeated more than four times in one day.

Herbal medications — There are a number of claims, particularly on the internet, that herbal medications can improve liver health. However, no single or combination of herbs has been proven to improve outcomes in people with AIH. Some herbs can cause serious liver damage, and some have been implicated in triggering AIH. For this reason, we do not currently recommend any herbal treatment for liver disease.

Support — Do not underestimate the value of sharing your concerns with other people with AIH. Ask your health care provider about support groups or ways to connect with other people who may be willing to discuss their experiences with AIH.

PREGNANCY AND AUTOIMMUNE HEPATITIS — People who are treated for autoimmune hepatitis (AIH) can have successful pregnancies. Treatment usually includes glucocorticoids and/or azathioprine, both of which are probably safe during pregnancy. (See "Patient education: Disease-modifying antirheumatic drugs (DMARDs) in rheumatoid arthritis (Beyond the Basics)".)

Stopping treatment during pregnancy can lead to relapse of the disease and is not usually recommended. People with AIH are also more likely to develop diabetes or have high blood pressure during pregnancy. Aspirin use may prevent blood-pressure-related complications and should be discussed with a health care provider before week 10 of pregnancy.

Babies of mothers with AIH may have an increased risk of prematurity, low birth weight, and other fetal problems. Pregnant people need to be monitored carefully during pregnancy and several months after delivery because of the risk of flares in disease activity.

LONG-TERM OUTCOME — Untreated AIH can cause scarring of the liver and ultimately lead to cirrhosis and liver failure. Fortunately, proper treatment can prevent scarring and cirrhosis in most people. Treatment can be beneficial, even if advanced scarring or cirrhosis has already developed, arresting progression of scarring and, sometimes, reversing the scarring.

Approximately 10 to 40 percent of people with AIH go into remission and no longer need medications for their condition; however, only approximately 20 to 30 percent of these people stay in remission. Thus, most people need either continuous therapy or additional rounds of medication to treat ongoing disease.

WHERE TO GET MORE INFORMATION — Your health care provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for health care professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

This topic currently has no corresponding Basic content.

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

Patient level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Patient education: Hepatitis A (Beyond the Basics)
Patient education: Hepatitis B (Beyond the Basics)
Patient education: Hepatitis C (Beyond the Basics)
Patient education: Cirrhosis (Beyond the Basics)
Patient education: Liver biopsy (Beyond the Basics)
Patient education: Disease-modifying antirheumatic drugs (DMARDs) in rheumatoid arthritis (Beyond the Basics)

Professional level information:

Overview of autoimmune hepatitis
Autoimmune hepatitis variants: Definitions and treatment
Autoimmune hepatitis: Pathogenesis
Management of autoimmune hepatitis

The following organizations also provide reliable health information.

●American Association for the Study of Liver Diseases

     (www.aasld.org)

●American Liver Foundation

     (www.liverfoundation.org)

●Hepatitis Foundation International

     (https://hepatitisfoundation.org/)

●National Institute of Diabetes and Digestive and Kidney Diseases

     (www.niddk.nih.gov)

●National Institutes of Health: Clinical Trials

     (www.clinicaltrials.gov)

●National Library of Medicine

     (www.nlm.nih.gov/medlineplus/healthtopics.html)