Primary Biliary Cholangitis (PBC) Profile (RDL)

PBC is an organ-specific autoimmune disease characterized by chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and ultimately fibrosis. PBC affects women more than men and typically occurs between the ages of 30 and 65. PBC affects women more than men and typically occurs between the ages of 30 and 65. The prevalence of PBC in first-degree relatives of PBC patients range is from 1.3 to 6.4%.

Anti-mitochondrial antibodies (AMA) have been reported in 90-96% of patients with PBC. AMA are also occasionally found in other liver conditions, including chronic active hepatitis, cryptogenic cirrhosis and in sera of individuals with clinical but no biochemical evidence of liver disease.

Serologic assays are important aids to the recognition and diagnosis of PBC since many antibodies associated with PBC are present before symptoms become evident. Anti-GP-210 and Anti-SP-100 antibodies have relatively low sensitivity, but specificity greater than 99% and either is detected in approximately 25% of all PBC patients and 30% of AMA-negative PBC patients. Additionally, these antibodies may identify a subgroup of patients with a more severe disease course.

Combined testing for the three markers, Anti-M2, Anti-GP- 210 and Anti-SP-100, identified in 92% of PBC patients.