autoimmune hepatitis


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Hepatitis, Autoimmune

 

Definition

A form of liver inflammation in which the body's immune system attacks liver cells.

Description

Autoimmunity causes the body's defense mechanisms to turn against itself. Many of the tissues in the body can be the target of such an attack. While one tissue type predominates, others may be involved in a general misdirection of immune activity, perhaps because the specific target antigen is present in differing quantities in each of the affected tissues. There seem to be hereditary causes for autoimmunity, since these diseases tend to run in families and have genetic markers. Among the more common diseases believed to fall within this category are rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis, and psoriasis.
The process of autoimmune disease is very similar to infectious disease and allergy, so that great caution is observed in placing a disorder in this class. Germs were found to cause several diseases originally thought to be autoimmune. Allergens cause others. Many more may be uncovered. Autoimmunity is often believed to originate with a virus infection. A chemical in the virus resembles a body chemical so closely that the immune system attacks both.
Autoimmune hepatitis is similiar to viral hepatitis, a disease of the liver. It can be an acute disease that kills over a third of its victims within six months, it can persist for years, or it can return periodically. Some patients develop cirrhosis of the liver which, over time, causes the liver to cease functioning.

Causes and symptoms

Symptoms of autoimmune hepatitis resemble those of other types of hepatitis. Patients who develop autoimmune hepatitis experience pain under the right ribs, fatigue and general discomfort, loss of appetite, nausea, sometimes vomiting and jaundice. In addition, other parts of the body may be involved and contribute their own symptoms.

Diagnosis

Extensive laboratory testing may be required to differentiate this disease from viral hepatitis. The distinction may not even be made during the initial episode. There are certain markers of autoimmune disease in the blood that can lead to the correct diagnosis if they are sought. In advanced or chronic cases a liver biopsy may be necessary.

Treatment

Autoimmune hepatitis is among the few types of hepatitis that can be treated effectively. Since treatment itself introduces problems in at least 20% of patients, it is reserved for the more severe cases. Up to 80% of patients improve with cortisone treatment, although a cure is unlikely. Another drug—azathioprine—is sometimes used concurrently. Treatment continues for over a year and may be restarted during a relapse. At least half the patients relapse at some point, and most will still continue to have progressive liver scarring.
If the liver fails, transplant is the only recourse.

Prognosis

In spite of treatment autoimmune hepatitis can re-erupt at any time, and may continue to damage and scar the liver. The rate of progression varies considerably from patient to patient.

Resources

Organizations

American Liver Foundation. 1425 Pompton Ave., Cedar Grove, NJ 07009. (800) 223-0179. http://www.liverfoundation.org.

Key terms

Allergen — Any chemical that causes an immune reaction only in people sensitive to it.
Antigen — Any chemical that can be the target of an immune response.
Biopsy — Surgical removal of a piece of tissue for examination.
Jaundice — A yellow color to the skin from bile that backs up into the circulation.

autoimmune hepatitis (AIH),

chronic liver disease of unknown etiology characterized by histologic presence of periportal hepatitis with plasma cell infiltration, hypergammaglobulinemia, and presence of autoantibodies in the serum. The disease has a female predilection and is highly responsive to immunosuppressive therapy.

autoimmune hepatitis

A multisystem disorder that primarily affects women of all ages, coexists with other liver diseases (e.g., chronic viral hepatitis) and is triggered by viral infections (e.g., HAV) and chemicals (e.g., minocycline).

Aetiology
Linked to circulating autoantibodies, and may be linked to other autoimmune disorders—e.g., thyroiditis, diabetes, ulcerative colitis, Coombs-positive haemolytic anaemia, proliferative glomerulonephritis, Sjögren syndrome.

Lab
Increased: IgG; anti-nuclear, anti-smooth muscle, anti-LKM, anti-mitochondrial antibodies; and anti-phospholipid antibodies; elevated 
LFTs.

Management
Daily prednisone.

Types of autoimmune hepatitis
• Type 1—Most common form of AIH in North America; 80% are women; may have increased anti-smooth muscle antibodies, anti-nuclear antibodies, anti-actin, and often have a marked increase in immune globulins.
• Type 2—Less common than type 1; affects children ages 2 to 14, 90% female; typically have anti-LKM antibodies.
• Type 3—Similar to type 1; 90% occur in younger (age 30–50) females.

autoimmune hepatitis

Lupoid hepatitis A type of chronic active hepatitis attributed to various circulating autoantibodies, which may be linked to other autoimmune diseases–eg, thyroiditis, DM, ulcerative colitis, Coombs-positive hemolytic anemia, proliferative glomerulonephritis, Sjögren syndrome
References in periodicals archive ?
Autoimmune hepatitis (AIH) is a chronic inflammatory condition of the liver with unknown etiology.
Porta et al., "Frequency of concurrent autoimmune disorders in patients with autoimmune hepatitis: effect of age, gender, and genetic background," Journal of Clinical Gastroenterology, vol.
Diagnostic criteria used for conventional AIH may not be applicable to cases with seronegative autoimmune hepatitis. Current report suggests that RDC is more helpful in diagnosing patients who are negative for conventional autoimmune serology.
Kanzler et al., "Transforming growth factor-[beta]1 in autoimmune hepatitis: correlation of liver tissue expression and serum levels with disease activity," Journal of Hepatology, vol.
You et al., "Interleukin-17 contributes to the pathogenesis of autoimmune hepatitis through inducing hepatic interleukin-6 expression," PLoS ONE, vol.
Hepatic involvement was mild and asymptomatic in our subjects, except in four lupus cases with overlapping autoimmune hepatitis. They were presenting with increased levels of alkaline phosphatase and gamma-glutamyltranspeptidase as well as specific autoimmune hepatitis antibodies.
Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly.
Prognosis of histological cirrhosis in type 1 autoimmune hepatitis. Gastroenterology 1996;110:848-57.
Autoimmune complications (AICs) after HSCT are still not well characterized but have multifactorial occurrence, such as autoimmune cytopenias (AICPs), autoimmune hepatitis (AIH), and autoimmune thyroid disease (AITD).
Hepatic involvement in SLE could be due to a wide range of factors such as drug induced damage, steatosis, viral hepatitis, vascular thrombosis, and overlaps with autoimmune hepatitis (AIH) or due to SLE itself.
Her particular hepatic metabolism further increased the drugs' concentration, favoring the haptenization of liver proteins, eventually leading to the development of an autoimmune hepatitis.
Excluded were other etiologies such as pre-existing autoimmune hepatitis, chronic alcohol use, and chronic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, Wilson's disease, and hemochromatosis.

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