144 - Autoimmune hepatitis
Autor(s): D. Villalta
Issue: RIMeL - IJLaM, Vol. 4, N. 2, 2008 (MAF Servizi srl ed.)
Page(s): 144-151
Autoimmune hepatitis (AIH) is a chronic progressive disease of unknown origin, characterized by interface hepatitis, hypergamaglobulinemia and tissue autoantibodies, which is responsive to immunosupressive therapy in most cases. Classically AIH is subdivided in two types on the basis of the immunological profile; type 1 AIH is characterized by anti nuclear (ANA) and anti smooth muscle (SMA) antibodies, while type 2 AIH is marked by antibodies to liver and kidney microsomes (LKM1) and liver cytosol type 1 antigen (LC1). Anti-soluble liverantigen (anti-SLA) antibodies were at the beginning associated with type 3 AIH, but it is now clear that most patients with anti-SLA in reality have either type 1 or type 2 AIH, and therefore type 3 AIH is not recognized by the International Autoimmune Hepatitis Group. Even if serum autoantibodies have a central role in the diagnosis and classification of AIH, they generally lack both of disease and tissue specificity and their pathogenetic involvement remains unclear. Finally, with the possible exceptions of anti-SLA, anti- LC1 and anti-asyaloglycoprotein receptor (anti-ASGPR) antibodies, serum autoantibody positivity and titer do not seem to associate with disease activity and/or outcome.