Autoimmune hepatitis

Autoimmune hepatitis

Author
Edward L Krawitt, MD
Section Editor
Sanjiv Chopra, MD
Deputy Editor
Peter A L Bonis, MD

Disclosures

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. In autoimmune hepatitis, the body’s immune system attacks the cells of the liver, which causes the liver to become inflamed.

Topics that discuss other types of hepatitis are available separately. (See “Patient information: Hepatitis A” and “Patient information: Hepatitis B” and “Patient information: Hepatitis C”.)

AUTOIMMUNE HEPATITIS CAUSES — It is not clear why autoimmune hepatitis 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 autoimmune hepatitis: type 1 and type 2.

Type 1 autoimmune hepatitis can affect people of any age or gender.
Type 2 autoimmune hepatitis primarily affects girls and young women and is less common.
There are also rare forms of autoimmune hepatitis (called variants) that have features of both autoimmune hepatitis and other liver diseases (primary sclerosing cholangitis or primary biliary cirrhosis).

AUTOIMMUNE HEPATITIS SYMPTOMS — Many patients with autoimmune hepatitis 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, autoimmune hepatitis can progress to cirrhosis (severe scarring of the liver). (See “Patient information: Cirrhosis”.)

AUTOIMMUNE HEPATITIS DIAGNOSIS — Autoimmune hepatitis is diagnosed with blood tests 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 l causes of liver disease. (See “Patient information: Liver biopsy”.)

AUTOIMMUNE HEPATITIS TREATMENT — Not everyone with autoimmune hepatitis needs treatment immediately. The decision to treat is based upon 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 (file://www.aasld.org/practiceguidelines/Pages/default.aspx).

Medications — Autoimmune hepatitis is usually treated first with a glucocorticoid (such as prednisone).

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.
Azathioprine or 6-mercaptopurine — A second medication, such as azathioprine {Azasan®; Imuran®} or 6-mercaptopurine (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.
Budesonide — Budesonide, another medication that may be substituted for prednisone, continues to be studied, but is not yet widely used.
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 autoimmune hepatitis 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. Men and women who use mycophenolate must use two effective methods to prevent pregnancy (eg, condoms and a birth control pill).

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. 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 [1]. 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.

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, and a liver biopsy is usually repeated at least every two years.

Self care — Taking medication and seeing a healthcare 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 patients with autoimmune hepatitis. 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 autoimmune hepatitis.

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. Patients 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 autoimmune hepatitis.

Prescription and nonprescription drugs — Many drugs are broken down by the liver. Thus, it is always best to check with a healthcare 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 (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 patients with autoimmune hepatitis. Some herbs can cause serious liver damage, and some have been implicated in triggering autoimmune hepatitis. 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 autoimmune hepatitis. Ask your healthcare provider about support groups or other patients who may be willing to discuss their experiences with autoimmune hepatitis.

PREGNANCY AND AUTOIMMUNE HEPATITIS — Women who are treated for autoimmune hepatitis can have successful pregnancies. Treatment usually includes glucocorticoids and/or azathioprine, both of which are probably safe during pregnancy. (See “Patient information: Disease modifying antirheumatic drugs (DMARDs)”.) Stopping treatment during pregnancy can lead to relapse of the disease, and is not usually recommended.

However, babies of women with AIH may have an increased risk of prematurity, low birth weight, and other fetal problems. Women 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 autoimmune hepatitis can cause scarring of the liver and ultimately lead to cirrhosis and liver failure. Fortunately, proper treatment can prevent scarring and cirrhosis in most patients. 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 autoimmune hepatitis go into remission and no longer need medications for their condition; however, only about 50 percent of these people stay in remission. Thus, most patients need either continuous therapy or additional rounds of medication to treat ongoing disease.

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

 

Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.

Patient level information:

Patient information: Hepatitis A
Patient information: Hepatitis B
Patient information: Hepatitis C
Patient information: Cirrhosis
Patient information: Liver biopsy
Patient information: Disease modifying antirheumatic drugs (DMARDs)

Professional level information:

Classification of autoimmune hepatitis
Clinical manifestations and diagnosis of autoimmune hepatitis
Definition and treatment of variants of autoimmune hepatitis
Extrahepatic manifestations of autoimmune hepatitis
Pathogenesis of autoimmune hepatitis
Serologic markers of autoimmune hepatitis
Treatment 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
(www.hepfi.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)

REFERENCES
Czaja AJ, Menon KV, Carpenter HA. Sustained remission after corticosteroid therapy for type 1 autoimmune hepatitis: a retrospective analysis. Hepatology 2002; 35:890.
Czaja AJ. Autoimmune liver disease. Curr Opin Gastroenterol 2004; 20:231.
Czaja AJ, Bianchi FB, Carpenter HA, et al. Treatment challenges and investigational opportunities in autoimmune hepatitis. Hepatology 2005; 41:207.
Manns MP, Czaja AJ, Gorham JD, et al. Diagnosis and management of autoimmune hepatitis. Hepatology 2010; 51:2193.
Krawitt EL. Autoimmune hepatitis. N Engl J Med 2006; 354:54.
Schramm C, Herkel J, Beuers U, et al. Pregnancy in autoimmune hepatitis: outcome and risk factors. Am J Gastroenterol 2006; 101:556.
Heneghan MA, Norris SM, O’Grady JG, et al. Management and outcome of pregnancy in autoimmune hepatitis. Gut 2001; 48:97.