Laboratory diagnosis of primary biliary cholangitis

Abstract

Primary biliary cholangitis (PBC) is the most common disease among autoimmune liver diseases. PBC is a chronic cholestatic disease with a progressive course which may extend over many decades. The disease offten has insidious onset and is quite often misdiagnosed or results in a prolonged course. Antimitochondrial autoantibodies (AMA) are the classical specific serological marker of PBC and are found in 90% to 95% of PBC patients. Antibodies to glycoprotein 210 (gp210) and nuclear autoantigen Sp 100 (sp100) are specific for PBC but with low sensitivity. They are more useful for dignosising AMA-negative PBC. An association between anti-gp210 antibodies and disease severity has been reported. AMA may be detectable in serum when patients are symptom-free and liver function tests are normal. Screening the patients during the early stage of the disease process, can influence the clinical phenotype, and long-term prognosis of PBC, and improve the quality of patient′s life. Key words: Autoimmune liver diseases; Primary biliary cholangitis; Antimitochondrial autoantibody