Autoimmune hepatitis
From Wikipedia, the free encyclopedia
| Autoimmune hepatitis Classification and external resources |
|
| ICD-10 | K75.4 |
|---|---|
| ICD-9 | 571.49 |
| DiseasesDB | 1150 |
| MedlinePlus | 000245 |
| eMedicine | med/366 |
| MeSH | D019693 |
Anomalous presentation of human leukocyte antigen (HLA) class II on the surface of hepatocytes,[citation needed] possibly due to genetic predisposition or acute liver infection; causes a cell-mediated immune response against the body's own liver, resulting in autoimmune hepatitis.
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%).[1] Liver enzymes are elevated, as may be bilirubin. Autoimmune hepatitis can progress to cirrhosis.
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[edit] Treatment
Treatment is with steroids and immunosuppressive drugs such as azathioprine and remission can be achieved in up to 60-80% of cases.[2]
[edit] Diagnosis
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), 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.[3][4]
[edit] Classification
Four subtypes are recognised, but the clinical utility of distinguishing subtypes is limited.
- positive ANA and SMA, raised immunoglobulin G (classic form, responds well to low dose steroids);
- positive LKM-1 (typically female children and teenagers; disease can be severe);
- all antibodies negative, positive antibodies against soluble liver antigen (SLA)(now designated SLP/LP) (this group behaves like group 1), and
- no autoantibodies detected (~20%)
[edit] References
- ^ Autoimmune Hepatitis.
- ^ Krawitt EL (January 1994). "Autoimmune hepatitis: classification, heterogeneity, and treatment". Am. J. Med. 96 (1A): 23S–26S. PMID 8109584.
- ^ Alvarez F, Berg PA, Bianchi FB, et al (November 1999). "International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis". J. Hepatol. 31 (5): 929–38. PMID 10580593.
- ^ Autoimmune Hepatitis Calculator. Retrieved on 2008-05-09.
