Autoimmune hepatitis (patient information)

Autoimmune hepatitis

Overview

What are the symptoms?

What are the causes?

Diagnosis

When to seek urgent medical care?

Treatment options

Where to find medical care?

Prevention

What to expect (Outlook/Prognosis)?

Possible complications

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief:  Manpreet Kaur, MD [2]

## Overview

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Autoimmune hepatitis is a chronic or long-lasting disease in which the body's immune system attacks the normal components, or cells, of the liver and causes inflammation and liver damage. The immune system normally protects people from infection by identifying and destroying bacteria, viruses, and other potentially harmful foreign substances. Autoimmune hepatitis is a serious condition that may worsen over time if not treated. Autoimmune hepatitis can lead to cirrhosis and liver failure. Cirrhosis occurs when scar tissue replaces healthy liver tissue and blocks the normal flow of blood through the liver. Liver failure occurs when the liver stops working properly.

## What are the symptoms of Autoimmune hepatitis?

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The most common symptoms of autoimmune hepatitis are:

* Fatigue
* Joint pain
* Nausea
* Loss of appetite
* Abdominal pain
* Skin Rashes
* Dark yellow urine
* Light-colored stools
* Jaundice, or yellowing of the skin

Symptoms of autoimmune hepatitis range from mild to severe. Some people may feel as if they have a mild case of the flu. Others may have no symptoms when a health care provider diagnoses the disease; however, they can develop symptoms later.

## What are the causes?

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A combination of autoimmunity, environmental triggers, and a genetic predisposition can lead to autoimmune hepatitis.

## When to seek urgent medical care?

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Call your health care provider if:

* You develop symptoms of autoimmune hepatitis.
* You have autoimmune hepatitis and the symptoms continue despite treatment.

## Diagnosis

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* A health care provider will make a diagnosis of autoimmune hepatitis based on symptoms, a physical exam, blood tests, and a liver biopsy.
* A health care provider performs a physical exam and reviews the person's health history, including the use of alcohol and medications that can harm the liver. A person usually needs blood tests for an exact diagnosis because a person with autoimmune hepatitis can have the same symptoms as those of other liver diseases or metabolic disorders.

### Blood tests

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A blood test involves drawing blood at a health care provider's office or a commercial facility and sending the sample to a laboratory for analysis. A person will need blood tests for autoantibodies to help distinguish autoimmune hepatitis from other liver diseases that have similar symptoms, such as viral hepatitis, primary biliary cirrhosis, steatohepatitis, or wilson disease.

#### Liver biopsy

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* A liver biopsy is a procedure that involves taking a piece of liver tissue for examination with a microscope for signs of damage or disease. The healthcare provider may ask the patient to temporarily stop taking certain medications before the liver biopsy. He or she may also ask the patient to fast for 8 hours before the procedure.
* During the procedure, the patient lies on a table, right hand resting on the head. A health care provider will apply a local anesthetic to the area where he or she will insert the biopsy needle. If needed, he or she will give sedatives and pain medication. Then, he or she will use a needle to take a small piece of liver tissue and may use ultrasound, computerized tomography scans, or other imaging techniques to guide the needle. After the biopsy, the patient must lie on the right side for up to 2 hours and is monitored an additional 2 to 4 hours before being sent home.
* A health care provider performs a liver biopsy at a hospital or an outpatient center. The liver sample is sent to a pathology lab where the pathologist—a doctor who specializes in diagnosing diseas looks at the tissue with a microscope and sends a report to the patient's health care provider.
* A health care provider can use the liver biopsy to diagnose autoimmune hepatitis and determine if cirrhosis is present. People often have cirrhosis at the time they are diagnosed with autoimmune hepatitis. A health care provider can also use liver biopsy to look for changes in the severity of liver damage prior to ending treatment for autoimmune hepatitis.

## Treatment options

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Treatment for autoimmune hepatitis includes medication to suppress or slow down, an overactive immune system. Treatment may also include a liver transplant.

Treatment works best when autoimmune hepatitis is diagnosed early. People with autoimmune hepatitis generally respond to standard treatment and the disease can be controlled in most cases. Long-term response to treatment can stop the disease from getting worse and may even reverse some damage to the liver.

Medications:

People with autoimmune hepatitis who have no symptoms or a mild form of the disease may or may not need to take medication. A health care provider will determine if a person needs treatment. In some people with mild autoimmune hepatitis, the disease may go into remission. Remission is a period when a person is symptom-free and blood tests and liver biopsy show improvement in liver function.

Corticosteroids

Corticosteroids are medications that decrease swelling and reduce the activity of the immune system. Health care providers treat both types of autoimmune hepatitis with a daily dose of a corticosteroid called prednisone. Treatment may begin with a high dose that is gradually lowered as the disease is controlled. The treatment goal is to find the lowest possible dose that helps control the disease.

Side effects of prednisone may include:

* Weight gain
* Thinning of the hair and skin
* Acne
* Diabetes
* High blood pressure
* Cataracts, a clouding in the lens of the eyes
* Glaucoma, elevated pressure in the eyes
* Anxiety and confusion

A health care provider will closely monitor and manage any side effects that may occur, as high doses of prednisone are often prescribed to treat autoimmune hepatitis.

Immune system suppressors. Medications that suppress the immune system prevent the body from making autoantibodies and block the immune reaction that contributes to inflammation. In most cases, health care providers use azathioprine (Azasan, Imuran) in conjunction with prednisone to treat autoimmune hepatitis. When using azathioprine, a health care provider can use a lower dose of prednisone, which may reduce prednisone's side effects.

Side effects of azathioprine include:

* Low white blood cell count
* Nausea
* Vomiting
* Skin rash
* Pancreatitis, or inflammation of the pancreas

Azathioprine is an immune system suppressor, so people taking the medication should undergo routine blood tests to monitor their white blood cell counts. A low white blood cell count can lead to bone marrow failure. Bone marrow is the tissue found in bones that produce new blood cells, including platelets. A health care provider will also check the platelet count when blood tests are done.

A person may need to discontinue prednisone or azathioprine if they cause severe side effects. The risk of side effects is higher in people who also have cirrhosis.

A healthcare provider may gradually reduce the dose of medication in people who show improvement, although the symptoms can return. When a person discontinues treatment, a health care provider will perform routine blood tests and carefully monitor the person's condition for a return of symptoms. Treatment with low doses of prednisone or azathioprine may be necessary on and off for many years.

People who do not respond to standard immune therapy or who have severe side effects from the medications may benefit from other immunosuppressive agents such as mycophenolate mofetil (CellCept), cyclosporine, or tacrolimus (Hecoria, Prograf).

Medications that suppress the immune system may lead to various forms of cancer. People on low doses of azathioprine for long periods of time are at slight risk of developing cancer.

Liver Transplant In some people, autoimmune hepatitis progresses to cirrhosis and end-stage liver failure, and a liver transplant may be necessary. Liver transplant is surgery to remove a diseased or an injured liver and replace it with a healthy one from another person, called a donor. A team of surgeons performs a liver transplant in a hospital. When possible, the patient fasts for 8 hours before the surgery. The patient stays in the hospital about 1 to 2 weeks to be sure the transplanted liver is functioning properly. The health care provider will monitor the patient for bleeding, infections, and signs of liver rejection. The patient will take prescription medications long term to prevent infections and rejection. Liver transplant surgery for autoimmune hepatitis is successful in most cases.

## Where to find medical care for autoimmune hepatitis?

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Medical care for autoimmune hepatitis can be found here.

## Prevention

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* There are no established measures for the secondary prevention of autoimmune hepatitis.
* There are few measures for the patient with autoimmune hepatitis should take to prevent complications:
* Patients with cirrhosis secondary to autoimmune hepatitis lead to ascites. Following recommendations are useful:
* A low-salt diet < 2gm of sodium daily is useful.
* Patients should consume protein (ie, >1.3 g protein per kg body weight).

## What to expect (Outlook/Prognosis)?

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Prognosis is generally excellent, and the 10-year survival rate of patients with autoimmune hepatitis treated with immunosuppressive therapy is approximately 80%.[1][2]

* Prognosis is poor without treatment, 50% of patients with severe autoimmune hepatitis will die in a 5 years.
* The presence of young age at presentation, AIH-2, coagulopathy, severe histologic activity is associated with a poor prognosis among patients with autoimmune hepatitis.

## Possible complications

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Patients can develop complications like cirrhosis, portal hypertension,esophageal varices, metabolic bone disease, hyperlipidaemia, hypovitaminosis and Cholestasis.

## Sources

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https://www.niddk.nih.gov/health-information/liver-disease/autoimmune-hepatitis

1. ↑ Hoeroldt B, McFarlane E, Dube A, Basumani P, Karajeh M, Campbell MJ, Gleeson D (2011). "Long-term outcomes of patients with autoimmune hepatitis managed at a nontransplant center". Gastroenterology. 140 (7): 1980–9. doi:10.1053/j.gastro.2011.02.065. PMID 21396370.
2. ↑ D'Amico G, Garcia-Tsao G, Pagliaro L (2006). "Natural history and prognostic indicators of survival in cirrhosis: a systematic review of 118 studies". J. Hepatol. 44 (1): 217–31. doi:10.1016/j.jhep.2005.10.013. PMID 16298014.