Autoimmune regulator AIRE: Evidence for genetic differences between autoimmune hepatitis and hepatitis as part of the autoimmune polyglandular syndrome type 1
Arndt Vogel, Heike Liermann, Andrea Harms, Christian P. Strassburg, Michael P. Manns, Petra Obermayer‐Straub – 30 December 2003 – The mechanisms driving the immune‐mediated destruction of hepatic tissues in autoimmune hepatitis (AIH) are unknown. Recently the autoimmune regulator (AIRE), a gene associated with the development of the autoimmune polyglandular syndrome type 1 (APS‐1), was cloned. About 15% to 20% of APS‐1 patients develop hepatitis. However, the role of AIRE mutations in AIH, primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) is not known.