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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.
Treatment is with steroids and immunosuppressive drugs such as azathioprine and remission can be achieved in up to 60-80% of cases.[2]
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]
Overlapping presentation with primary biliary cirrhosis and primary sclerosing cholangitis has been observed.[5]
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