Abstract
Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease of presumed autoimmune etiology, characterized by the destruction of small intrahepatic bile ducts and the eventual development of cirrhosis and liver failure. Patients are often seen associated with various collagen diseases, although details of this association remain unclear. In the current study, the presence of serum anti-mitochondrial antibodies (AMA), a hallmark of PBC, was determined among 302 collagen disease patients, and its clinical significance investigated. AMA were screened by immunofluorescence and ELISA, and subsequently analyzed by Western blot. Fourteen sera (4.6%) were found to be positive for AMA. Western blot analysis revealed that while only six of these sera reacted positive for anti-PDC-E2, 10 were positive for anti-BCOADC-E2. AMA had previously been detected in only 5 of these 14 cases. Furthermore, six of these patients were also found to be positive for anti-centromere antibody. In conclusion, a potential association with PBC should be considered in the medical examination and treatment of patients with collagen diseases.
