Autoimmune hepatitis: Difference between revisions
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==References== |
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* [http://www.torontoliver.ca/twhlc/en/education/autoimmune.html Patient Information Leaflets] - Autoimmune Liver Disease Education (Gideon Hirschfield) |
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Revision as of 13:49, 15 July 2011
| Autoimmune hepatitis | |
|---|---|
| Specialty | Gastroenterology, hepatology  |
Autoimmune Hepatitis is a disease of the liver that occurs when the body's immune system attacks cells of the liver. Anomalous presentation of human leukocyte antigen (HLA) class II on the surface of hepatocytes,[citation needed] possibly due to genetic penisposition or acute liver infection, causes a cell-mediated immune response against the body's own liver, resulting in autoimmune hepatitis. This abnormal immune response results in inflammation of the liver, which can lead to further complications, including cirrhosis.[1]
Autoimmune hepatitis has an incidence of 1-2 per 100,000 per year, and a prevalence of 10-20/100,000. As with most other autoimmune diseases, it affects women much more often than men (70%).[2] Liver enzymes are elevated, as may be bilirubin.
Classification
Four subtypes are recognised, but the clinical utility of distinguishing subtypes is limited.
- positive ANA and SMA,[3] raised immunoglobulin G (classic form, responds well to low dose steroids);
- positive LKM-1 (typically female children and teenagers; disease can be severe);
- positive antibodies against soluble liver antigen[4] (this group behaves like group 1)[5]
- no autoantibodies detected (~20%)[citation needed]
Diagnosis
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The diagnosis of autoimmune hepatitis is best achieved with a combination of clinical, laboratory and histological findings.
A number of specific antibodies found in the blood (antinuclear antibody (ANA), anti-Smooth Muscle Antibody (SMA), liver/kidney microsomal antibody (LKM-1), anti soluble liver antigen (SLA/LP) and anti-mitochondrial antibody (AMA)) are of use, as is finding an increased Immunoglobulin G level. However, the diagnosis of autoimmune hepatitis always requires a liver biopsy.
In complex cases, a scoring system can be used to help determine if a patient has autoimmune hepatitis, which combines clinical and laboratory features of a given case.[6][7]
Overlapping presentation with primary biliary cirrhosis and primary sclerosing cholangitis has been observed.[8]
Treatment
Treatment is with glucocorticoids with or without azathioprine and remission can be achieved in up to 60–80% of cases, although many will eventually experience a relapse.[9] Patients who do not respond to glucocorticoids and azathioprine may be given other immunosuppressives like cyclosporin, tacrolimus, methotrexate etc. Liver transplantation may be required if patients do not respond to drug therapy or when patients present with fulminant liver failure.[10]
References
- ^ National Digestive Diseases Information Clearinghouse. "Digestive Disease: Autoimmune Hepatitis". Retrieved October 9, 2010.,
- ^ "Autoimmune Hepatitis".
- ^ Bogdanos DP, Invernizzi P, Mackay IR, Vergani D (2008). "Autoimmune liver serology: Current diagnostic and clinical challenges". World J. Gastroenterol. 14 (21): 3374–3387. doi:10.3748/wjg.14.3374. PMC 2716592. PMID 18528935.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) CS1 maint: unflagged free DOI (link) - ^ "autoimmune hepatitis".
- ^ "Medscape & eMedicine Log In".
- ^ Alvarez F, Berg PA, Bianchi FB; et al. (1999). "International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis". J. Hepatol. 31 (5): 929–38. doi:10.1016/S0168-8278(99)80297-9. PMID 10580593. Retrieved 2008-05-09.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ "Autoimmune Hepatitis Calculator". Retrieved 2008-05-09.
- ^ Washington MK (2007). "Autoimmune liver disease: overlap and outliers". Mod. Pathol. 20 Suppl 1: S15–30. doi:10.1038/modpathol.3800684. PMID 17486048.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Krawitt EL (1994). "Autoimmune hepatitis: classification, heterogeneity, and treatment". Am. J. Med. 96 (1A): 23S–26S. doi:10.1016/0002-9343(94)90186-4. PMID 8109584.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Stephen J Mcphee, Maxine A Papadakis. Current medical diagnosis and treatment 2009 page.596
External links
- Patient Information Leaflets - Autoimmune Liver Disease Education (Gideon Hirschfield)