Chloride/bicarbonate exchange in PBC: A clue for pathogenesis?

摘要: Primary biliary cirrhosis (PBC), a chronic cholestatic process affecting mainly women, is currently considered to be an autoimmune disease with humoral and cellular immunity directed against mitochondrial antigens or crossreactive epitopes1,2. However, the molecular changes which originate reaction mechanisms responsible for altered immunoregulation in PBC are unknown. Many questions concerning pathogenesis of this remain answered. For example, there patients virtually identical who do not present antimitochondrial antibodies. Also, cases antibodies develop after initiation pathological alterations liver. It also intriguing that ursodeoxycholic acid (UDCA), bile induces bicarbonaterich choleresis, affords more therapeutic benefit than classical immunosuppressants despite involved disease3. Moreover, no explanation functional abnormal distribution cell observed isolated epithelial cells (BEC) from patients.