<50% survival if untreated, >90% survival if treated
Frequency
Incidence 1-2 per 100,000 per year Prevalence 10-25 per 100,000
Autoimmune hepatitis, formerly known as lupoid hepatitis, plasma cell hepatitis, or autoimmune chronic active hepatitis, is a chronic, autoimmune disease of the liver that occurs when the body's immune system attacks liver cells, causing the liver to be inflamed. Common initial symptoms may include fatigue, nausea, muscle aches, or weight loss or signs of acute liver inflammation including fever, jaundice, and right upper quadrant abdominal pain. Individuals with autoimmune hepatitis often have no initial symptoms and the disease may be detected by abnormal liver function tests and increased protein levels during routine bloodwork or the observation of an abnormal-looking liver during abdominal surgery.[1]
Anomalous presentation of MHC class II receptors on the surface of liver cells,[2] 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. This abnormal immune response results in inflammation of the liver, which can lead to further symptoms and complications such as fatigue and cirrhosis.[3] The disease is most often diagnosed in patients in their late teens or early 20s and between the ages of 40 and 50. It affects women more commonly than men.[4]
^Czaja AJ (May 2004). "Autoimmune liver disease". Current Opinion in Gastroenterology. 20 (3): 231–240. doi:10.1097/00001574-200405000-00007. PMID 15703647.
^Franco A, Barnaba V, Natali P, Balsano C, Musca A, Balsano F (May–June 1988). "Expression of class I and class II major histocompatibility complex antigens on human hepatocytes". Hepatology. 8 (3): 449–454. doi:10.1002/hep.1840080302. PMID 2453428. S2CID 23341082.
^National Digestive Diseases Information Clearinghouse. "Digestive Disease: Autoimmune Hepatitis". Archived from the original on 15 September 2010. Retrieved October 9, 2010.
^Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D, Vierling JM (June 2010). "Diagnosis and management of autoimmune hepatitis". Hepatology. 51 (6): 2193–2213. doi:10.1002/hep.23584. PMID 20513004. S2CID 30356212.
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