Autoimmune Hepatitis

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What is autoimmune hepatitis?

Autoimmune hepatitis is a type of liver inflammation in which the body’s immune cells attack healthy liver cells after mistaking them for disease-causing foreign substances. It affects children and adults at any age. It is often diagnosed in individuals who have been previously diagnosed with a different autoimmune disorder.

The liver assists the digestive system and carries out many other essential functions. These functions include producing bile to help break down food into energy; creating essential substances, such as hormones; cleaning toxins from the blood, including those from medication, alcohol and drugs; and controlling fat storage and cholesterol production and release.

Autoimmune hepatitis is a disease that affects two body systems, the liver, which is part of your gastrointestinal system, and your immune system. While other forms of hepatitis are caused by bacteria or viruses, in autoimmune hepatitis, the immune system itself attacks the liver cells because it cannot distinguish between harmful invaders and healthy liver tissue.

The prognosis for autoimmune hepatitis varies. In many people, corticosteroid therapy is effective in slowing or even stopping the disease’s progress. In other cases, autoimmune hepatitis may develop into cirrhosis, in which liver cells are replaced with scar tissue. Cirrhosis causes permanent liver dysfunction and may require a liver transplant. Patients with chronic liver inflammation are at increased risk for developing liver cancer.

All symptoms of autoimmune hepatitis require prompt attention, but some are more urgent than others. Seek immediate medical care (call 911) for serious symptoms associated with complications, such as confusion, agitation, severe abdominal swelling, severe abdominal pain, sweating, and severe difficulty breathing.

Seek prompt medical care if you experience abdominal swelling, dark urine, fatigue, malaise, itchy skin, loss of appetite, nausea with or without vomiting, or pale or clay-colored stools. Also seek prompt medical care if you are being treated for autoimmune hepatitis, but your symptoms recur or are persistent.

What are the symptoms of autoimmune hepatitis?

Symptoms of autoimmune hepatitis involve a variety of body systems, and the effects can range from reduced energy level to skin irritation to abdominal and gastrointestinal symptoms. There can be marked variability in symptom intensity: near normal to extreme. You should seek medical attention for any one of these symptoms and seek immediate attention for symptoms associated with more serious stages and complications.

Gastrointestinal symptoms of autoimmune hepatitis

You may experience autoimmune hepatitis symptoms daily or not at all until the disease is more advanced. Some symptoms of autoimmune hepatitis directly affect the function of the gastrointestinal system. At times any of these symptoms can be severe:

  • Abdominal swelling, distention or bloating

  • Loss of appetite

  • Nausea with or without vomiting

  • Pale or clay-colored stools

  • Yellowing of the skin and whites of the eyes (jaundice)

Additional symptoms of autoimmune hepatitis

In autoimmune hepatitis, symptoms affecting other parts of the body can be just as prominent as those that more directly affect the digestive system. At times any of these symptoms can be severe:

  • Dark urine

  • Fatigue

  • Itchy skin

  • Spider angiomas (red skin lesions consisting of small blood vessels that spread out in a pattern that resembles a spider web)

Serious symptoms that might indicate a life-threatening condition

In some cases, autoimmune hepatitis can be life threatening. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms including:

What causes autoimmune hepatitis?

In autoimmune diseases, the immune system itself initiates inflammation because it cannot tell the difference between harmful invaders and healthy body tissue. Thus, in the case of autoimmune hepatitis, the body’s immune cells attack healthy cells in the liver after mistaking them for disease-causing foreign substances.

Autoimmune hepatitis is sometimes classified as type 1 or type 2. Type 1 is far more common and can occur along with other autoimmune diseases, such as Graves’ disease (type of hyperthyroidism resulting in excessive thyroid hormone production); inflammatory bowel disease; rheumatoid arthritis (chronic autoimmune disease characterized by joint inflammation); scleroderma (disorder characterized by thickening and stiffening of the skin); Sjogren’s syndrome (chronic autoimmune disease characterized by dry eyes and dry mouth); systemic lupus erythematosus (disorder in which the body attacks its own healthy cells and tissues); Hashimoto’s thyroiditis (autoimmune disease resulting in hypothyroidism and low production of thyroid hormone); type 1 diabetes; or ulcerative colitis.

Autoimmune hepatitis also sometimes occurs in people who have relatives with autoimmune diseases, leading scientists to suspect a possible genetic cause. Type 2 autoimmune hepatitis is most common in girls and young women.

What are the risk factors for autoimmune hepatitis?

Several factors seem to increase the risk of developing autoimmune hepatitis. You may be at a slightly higher risk if you are a girl or young woman, if you have a relative with any autoimmune disorder, or if you already have an autoimmune disorder. Not all people with risk factors will get autoimmune hepatitis. Risk factors for autoimmune hepatitis can include:

  • Family history of an autoimmune disorder

  • Female sex (fairly low risk rate)

Your risk of autoimmune hepatitis is also increased if you have another autoimmune disease including:

  • Graves’ disease (type of hyperthyroidism resulting in excessive thyroid hormone production)

  • Hashimoto’s thyroiditis (autoimmune disease resulting in hypothyroidism and low production of thyroid hormone)

  • Rheumatoid arthritis (chronic autoimmune disease characterized by joint inflammation)

  • Scleroderma (disorder characterized by thickening and stiffening of the skin)

  • Sjogren’s syndrome (chronic autoimmune disease characterized by dry eyes and dry mouth)

  • Systemic lupus erythematosus (disorder in which the body attacks its own healthy cells and tissues)

  • Type 1 diabetes

How is autoimmune hepatitis treated?

The treatment of autoimmune hepatitis begins with seeking medical care from your health care provider. If you have autoimmune hepatitis, your doctor will most likely start you on a course of corticosteroid therapy and, if needed, other immunosuppressants (medications that suppress the activity of the immune system). Following your treatment plan can slow or sometimes even stop the progression of this disease, and many people can have long, active lives with this disease. However, untreated or poorly treated autoimmune hepatitis may develop into cirrhosis, in which liver cells are replaced with scar tissue. Cirrhosis causes permanent liver dysfunction and may require a liver transplant.

Most common medications

Two medications stand out as first-round drugs of choice for long-term treatment to control disease progression. Many people experience successful long-term control, and even remission, with these agents. These agents may be used alone or in combination:

  • Azathioprine (Imuran), an immunosuppressant agent

  • Corticosteroids, such as prednisone (Deltasone, Orasone) and prednisolone (Prelone)

Other immunosuppressive agents

If corticosteroids and azathioprine are not working for you, your doctor may prescribe one of the following immunosuppressants, which are highly effective in some individuals. These include:

  • Cyclosporine (Gengraf, Neoral, Sandimmune)

  • Mycophenolate (Cellcept)

  • Tacrolimus (Prograf)

Surgical intervention

Autoimmune hepatitis can progress to end-stage liver disease or liver failure. Treatment for liver failure is liver transplant.

What are the potential complications of autoimmune hepatitis?

Complications of untreated or poorly controlled autoimmune hepatitis can be serious, even life threatening in some cases. You can help minimize your risk of serious complications by following the treatment plan you and your health care professional design specifically for you. Complications of autoimmune hepatitis include:

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Nov 24
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THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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