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=Autoimmune= improved |
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Anomalous presentation of [[human leukocyte antigen]] (HLA) class II on the surface of [[hepatocyte]]s — possibly due to [[gene]]tic predisposition or acute liver infection — causes a cell-mediated [[immune response]] against the body's own liver, resulting in autoimmune hepatitis.
''Autoimmune hepatitis'' has a prevalence of 1-2 per 1000. As with most other [[autoimmune disease]]s, it affects women much more often than men (8:1). [[Liver enzyme]]s are elevated, as is [[bilirubin]].
The diagnosis is confirmed by testing for antibodies: antinuclear antibody (ANA), smooth muscle antibody (SMA), Liver/kidney microsomal antibody (LKM-1) and anti-mitochondrial antibody (AMA). There is often an increased [[immunoglobulin]] level.
Four types are recognised:
# Positive ANA and SMA, raised immunoglobulin G
# Positive LKM-1 (typically children and teenagers; disease can be severe)
# All antibodies negative, positive antibodies against soluble liver antigen (SLA)
# No autoantibodies detected
Treatment is with [[steroid]]s and [[disease-modifying antirheumatic drug]]s (DMARDs). Chronic inflammation can progress to [[cirrhosis]].
=== A1AD ===
In severe cases of [[alpha 1-antitrypsin deficiency]] (A1AD), the acculumated [[protein]] causes in the [[endoplasmic reticulum]] causes liver cell damage and [[inflammation]]. This is technically not an autoimmune disease.
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