What Is Autoimmune Hepatitis C

What Is The Prognosis For Patients Who Have Autoimmune Hepatitis

If autoimmune hepatitis is diagnosed early, and treated with the proper medication, the liver may begin to heal and will be able to make healthy cells again to replace the inflamed and scarred cells. The patients symptoms will ease and the liver may begin to work normally.

The patient will need to be monitored and managed for the rest of his or her life, even if he or she is feeling better and liver function has improved. In many cases, the patient will need to be on medication for the rest of his or her life.

Last reviewed by a Cleveland Clinic medical professional on 06/21/2018.

References

Getting Tested Is The Only Way To Know If You Have Hepatitis C

A blood test called a hepatitis C antibody test can tell if you have been infected with the hepatitis C viruseither recently or in the past. If you have a positive antibody test, another blood test is needed to tell if you are still infected or if you were infected in the past and cleared the virus on your own.

• Are 18 years of age and older
• Are pregnant
• Currently inject drugs
• Have ever injected drugs, even if it was just once or many years ago
• Have HIV
• Have abnormal liver tests or liver disease
• Are on hemodialysis

Is There A Cure

Not yet. Since we do not know exactly what causes autoimmune hepatitis, we do not yet have a medical cure for the condition. Steroids and other anti-inflammatory drugs can certainly control the inflammation in the majority of cases. However, in certain patients in whom the inflammation continues or who have not been detected and diagnosed until very late in the course, cirrhosis can occur. Cirrhosis is a condition characterized by increased scar tissue that destroys the normal architecture of the liver.

Even if cirrhosis occurs, patients who have a mild disease without active inflammation generally do well and can live many years or decades without problems. If inflammation continues, the cirrhosis usually worsens, eventually reaching a stage called end-stage liver failure. If this stage has been reached, liver transplantation can be used in some patients to successfully treat the condition.

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Dos And Donts In Managing Autoimmune Hepatitis:

• DO remember that monitoring of your condition is important. Report any new symptoms to your health care provider promptly.
• DO call your health care provider if you notice skin color changes, side effects from medicines, joint pains, or abdominal swelling.

• DONT ignore drug side effects, such as weight gain, anxiety, confusion, thinning of bones , thinning of the hair and skin, diabetes, high blood pressure, and cataracts.
• DONT use alcohol. It may further damage your liver.

• National Institute of Diabetes and Digestive and Kidney Disease

Do Medicines Used To Treat Autoimmune Hepatitis Have Side Effects

Medicines for autoimmune hepatitis can cause side effects. Your doctor will monitor any side effects and help you manage them while you take these medicines. Your doctor also may adjust the doses or change the medicines you take. You may need to stop taking corticosteroids or azathioprine if you have severe side effects.

Side effects of corticosteroids may include

• changes in how you look, which may include weight gain, a fuller face, acne, or more facial hair

• liver damage
• pancreatitis

Corticosteroids and azathioprine suppress, or decrease the activity of, your immune system, which increases your risk for infections. These medicines can also increase your risk of developing cancers, especially skin cancers.

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What Are The Mortality Risk Factors Of Autoimmune Hepatitis

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Without treatment, nearly 50% of patients with severe autoimmune hepatitis will die in approximately 5 years, and most patients will die within 10 years of disease onset. Treatment with corticosteroids has been shown to improve the chances of survival significantly. The 10-year life expectancies for treated patients with and without cirrhosis at presentation are 89% and 90%, respectively. Indeed, the life expectancy of patients in clinical remission is similar to that of the general population.

The Role Of Liver Biopsy In Aih

Liver biopsy is recommended by the American Association for the Study of Liver diseases and the European Association for the Study of the Liver guidelines to help establish the diagnosis, exclude other causes of liver disease, and guide treatment choice ,.

The diagnostic criteria for AIH have been codified in 1993, revised in 1999 by the International Autoimmune Hepatitis Group , and more recently simplified for clinical use . In the simplified system, as in the previous ones, liver histology is included among the parameters required to confirm clinical diagnosis of AIH. Indeed, the system comprises four parameters: autoantibodies, serum IgG, results of viral hepatitis work-up and AIH histology, which is coded as absent, typical or compatible .

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Drug Induced Liver Injury

Drug induced liver injury represents the most challenging differential diagnosis, not only because it can mimic the clinical, biochemical, serological and morphological phenotype of AIH , but also because drugs may trigger latent or induce a de novo AIH . The distinction between DILI and AIH by histology can be extremely difficult , due to the absence of histological features pathognomonic of either DILI or AIH. Severe portal plasma cell-rich inflammation, prominent intralobular plasmacells and eosinophils, rosette formation, absence of cholestasis and presence of fibrosis have been suggested as features that are in favor of the diagnosis of AIH . The absence of cirrhosis, or advanced fibrosis, at presentation mainly suggests AIH-like DILI. A detailed clinical information is crucial, and the patients history should focus on recent exposure to drugs that can induce AIH-DILI. Fortunately, AIH-DILI usually responds to high doses of steroids as severe AIH usually does, but differently from true AIH that always relapses, steroid treatment can be discontinued without a DILI relapse .

Hepatitis C Virus Antibody In Patients With Chronic Autoimmune Hepatitis: Pitfalls In Diagnosis And Implications For Treatment

• Gary L. DavisCorrespondenceAddress reprint requests to Dr. G. L. Davis, Box J-214, J. Hillis Miller Health Center, University of Florida, Gainesville, FL 32610-0214AffiliationsDirector, Hepatobiliary Section Division of Gastroenterology, Hepatology, and Nutrition University of Florida College of Medicine Gainesville, Florida

ProceedingsWhy is the occurrence of anti-HCV in patients with autoimmune hepatitis or the presence of antinuclear antibodies in patients with chronic hepatitis C a problem?

How should the clinician approach the patient in whom the distinction between autoimmune hepatitis and hepatitis C is unclear?

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Treatments For Autoimmune Hepatitis

Treatment works best when AIH is diagnosed early. The goal in treating AIH is to slow or stop the bodys immune system from attacking the liver. The medications used are immunosuppressants, such as prednisone and Imuran® . Physicians usually prescribe a high initial dose of prednisone, and then taper it down progressively as symptoms and liver enzymes improve. Most people will need to take medication for the rest of their lives. Since prednisone can cause a wide range of side effects, Imuran® is often used in conjunction to allow for a lower dose of the prednisone.

Some people may go into remission, during which physicians can effectively discontinue treatment others will relapse after stopping treatment, and will then need to restart the medication and continue on long-term maintenance therapy. A few patients may eventually be tapered off the prednisone completely and stay solely on Imuran®. For those who do not respond to, or relapse from, the combination regimen, then stronger immunosuppressive agents such as mycophenolate mofetil, cyclosporine, or tacrolimus may be considered. When medications do not halt the progress of the disease, or complications from cirrhosis have developed, the remaining option is a liver transplant. Fortunately, the success rate of transplantation in people with AIH is excellent.

Is An Aih Diagnosis Fatal

If left untreated, autoimmune hepatitis could be fatal. Early diagnosis and treatment are essential to achieving a good prognosis.

For individuals who respond positively to treatment, the 10-year survival rate is about 83.8% to 94%. Without any treatment, 40% to 50% of individuals with severe autoimmune hepatitis will die within six months to five years.

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What Is The Outlook

With treatment, most people with autoimmune hepatitis have a normal life expectancy and feel well most of the time. The treatment used for autoimmune hepatitis has improved the outlook tremendously. It is very important that you do not stop your treatment too early without your doctor’s knowledge, as your hepatitis may return. Although the condition usually returns at some point after stopping treatment, it can usually be treated again by quickly going back on medication.

There is a very small increased risk of developing liver cancer, especially if you also have ‘scarring’ of the liver due to your autoimmune hepatitis. Some doctors recommend a blood test and an ultrasound scan of your liver every so often to screen for this.

How Do Doctors Treat Autoimmune Hepatitis

Doctors treat autoimmune hepatitis with medicines that suppress, or decrease the activity of, your immune system, reducing your immune systems attack on your liver. The medicines doctors most often prescribe are corticosteroidsprednisone or prednisolonewith or without another medicine called azathioprine.

Doctors typically start with a relatively high dose of corticosteroids and then gradually lower the dose. Your doctor will try to find the lowest dose that works for you. Your doctor will use blood tests to find out how you are responding to the treatment. A decrease in levels of the liver enzymes alanine transaminase and aspartate transaminase shows a response to treatment. ALT and AST falling to normal levels shows a full response. In some cases, a doctor may repeat a liver biopsy to confirm the response to treatment and find out whether the damage has resolved.

Treatment can relieve symptoms and prevent or reverse liver damage in many people with autoimmune hepatitis. Early treatment of autoimmune hepatitis can lower the chances of developing cirrhosis and other complications. A minority of people who have no symptoms or only a mild form of the disease may or may not need medicines.

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Overview On Laboratory And Clinical Features

Liver function tests typically show a hepatocellular pattern of injury, with an increase in aminotransferases, that can be mildly elevated or up to 50 times the upper normal value. Alanine aminotransferase is typically higher than Aspartate aminotransferase . Cholestatic enzymes are usually normal or mildly elevated unless there is an overlap with primary biliary cholangitis or primary sclerosing cholangitis . Increase in serum globulins is evident in 90% of patients. This prevalence seems to be lower in patients with an acute onset, among which a proportion of cases, ranging from 25 to 39%, has normal IgG levels ,. IgA and IgM are usually normal, and their increased levels should prompt the exclusion of different etiologies, such as alcoholic steatohepatitis and PBC, respectively.

What Is The Treatment For Autoimmune Hepatitis

Treatment is almost always needed. Early treatment can improve symptoms, reduce the risk of complications, and also greatly improve your outlook . Treatment aims to reduce inflammation and suppress the immune system with immunosuppressant medicines:

• Steroid medication is the usual first treatment. Steroids are good at reducing inflammation. A high dose is usually needed at first. The dose is then gradually reduced over a few weeks. The aim is to find the lowest dose needed to control the inflammation. The dose needed varies from person to person. See the separate leaflet called Oral Steroids for more detail.
• Azathioprine is an immunosuppressant medicine that works in a different way to steroids. It is usually used in addition to the steroid. A steroid plus azathioprine tends to work better than either alone. Also, the dose of steroid needed is usually less if you also take azathioprine. This means that any side-effects from steroids may be less severe.

Treatment works well in most cases. Usually, the inflammation settles and symptoms improve within a few months of starting treatment. However, it may take a year or more to get the disease totally under control. Azathioprine is usually taken for at least two years.

For some people a liver transplant may be an option – for example:

• In the few people who do not respond to treatment with the medicines mentioned above or
• In people diagnosed in the late stage of the disease with severe ‘scarring’ of the liver or liver failure.

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How Is Autoimmune Hepatitis Treated

The goal of treatment is to stop the bodys attack on itself by suppressing the immune system. This is accomplished with a medicine called prednisone, a type of steroid. Often times, a second drug, azathioprine is also used. Treatment starts with a high dose of prednisone. When symptoms improve, the dosage is lowered and azathioprine may be added. In most cases, autoimmune hepatitis can be controlled but not cured. That is why most patients will need to stay on the medicine for years, and sometimes for life. Unfortunately, long-term use of steroid can cause serious side effects including diabetes, osteoporosis, high blood pressure, glaucoma, weight gain and decreased resistance to infection. Other medications may be needed to control these side effects.

When To Start Treatment

Treatment should be started in patients with significant disease, characterized by at least one of the following: AST or ALT > 10 times the upper limit of normal AST or ALT > 5 times the upper limit of normal and IgG > 2 times the upper limit of normal bridging necrosis or multiacinar necrosis on histology. Although uncommon, the presence of incapacitating symptoms has also been proposed as an indication of treatment regardless of laboratory values.

In asymptomatic patients with AST, ALT, and gamma globulins/IgG elevations that do not meet the criteria above, the benefit of treatment is less clear. The course of the disease in such patients has not been well established and there is little data to support treatment. Thus in asymptomatic patients with only mild laboratory and histological changes, the decision to start treatment should be individualized and the risks of therapy taken into account. Often treatment in this situation can be postponed and liver tests followed closely. Such patients should always be referred to a hepatologist or gastroenterologist for decision regarding therapy.

Asymptomatic patients with inactive disease on liver biopsy or burned out cirrhosis do not benefit from treatment.

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Quadrants And Regions Of Abdomen

The human is divided into quadrants and regions by and for the purposes of study, , and . The division into four quadrants allows the localisation of and , , lumps, and other items of interest, narrowing in on which and may be involved. The quadrants are referred to as the left lower quadrant, left upper quadrant, right upper quadrant and right lower quadrant. These terms are not used in , since most other animals do not stand erect.

The left lower quadrant includes the left and half of the . The equivalent in other animals is left posterior quadrant. The left upper quadrant extends from the to the left . This is the left anterior quadrant in other animals. The right upper quadrant extends from umbilical plane to the right ribcage. The equivalent in other animals is right anterior quadrant. The right lower quadrant extends from the umbilical plane to the right . This in other animals is the right posterior quadrant.

The nine regions offer more detailed anatomy and are delineated by two vertical and two horizontal lines.

Treatment For Autoimmune Hepatitis

Sometimes, autoimmune hepatitis goes away on its own. But if it does not resolve, prompt treatment is important to limit more serious problems such as cirrhosis and liver failure.

It can take several months of anti-inflammatory medicines before autoimmune hepatitis goes into remission. Some people may require lifelong medication to control the symptoms and prevent a recurrence.

If a liver transplant is necessary, our team works with the Johns Hopkins Comprehensive Transplant Center, known internationally for its innovative procedures and quality patient care.

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How To Prepare For Your First Appointment With A Hepatologist

Think through the questions that the doctor may ask you. Schedule some time to sit down and take notes before the appointment. Write down each of your symptoms and how long they have been going on. Think about any family members who have a history of liver disease or autoimmune conditions, and write them down as well. Finally, create a list of all of the medications, vitamins, and supplements you are currently taking.

Genetics And Predisposing Factors

Autoimmune hepatitis is thought to result from an environmental trigger in a genetically predisposed individual, leading to loss of tolerance of T lymphocytes with subsequent hepatocyte attack.

It is a polygenic disease and does not follow a Mendelian distribution. Therefore there is no need to screen family members of patients with AIH. There is a strong genetic association with the alleles of the major histocompatibility complex class II. The presence of human leukocyte antigen genes HLA DRB1*03 and HLA DRB1*04 predisposes to AIH type 1 and affect the disease course and response to treatment. Individuals who are positive for HLA DRB1*03 are younger, respond less favorably to corticosteroid therapy, and progress more often to liver failure. On the other hand, the presence of HLA DRB1*04 is associated with higher rates of concomitant autoimmune disorders.

Autoimmune hepatitis can also be associated with autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome, an autosomal recessive disease characterized by hypoparathyroidism, adrenal insufficiency, and chronic mucocutaneous candidiasis. Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy is the only AIH-associated disease that follows a Mendelian pattern of inheritance and genetic counseling should be offered for patients and family members.

Table 1: Drugs Associated With Drug-Induced Autoimmune-Like Hepatitis

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