La colangite autoimmune (CBP AMA negativa)

"Autoimmune cholangitis" is a term recently proposed to describe patients with clinical, biochemical, and histological features of primary biliary cirrhosis but lacking antimitochondrial antibodies, a serologie marker present in about 90% of patients with primary biliary cirrhosis. Although patients with "autoimmune cholangitis" have several peculiar serologie immunoreactivities and an anomalous expression of mitochondrial autoantigens at liver level, they seem not to differ from patients with antimitochondrial antibodies-positive primary biliary cirrhosis with respect to relevant biochemical, histological and clinicotherapeutic features. In this article we discuss in detail data supporting and data contrasting a new classification of chronic cholestatic liver diseases which include "autoimmune cholangitis" as a new clinical entity distinct from primary biliary cirrhosis.