Abstract: Primary biliary cholangitis (PBC) is a progressive cholestatic liver disease characterized
serologically by cholestasis and specific autoimmune antibodies and histologically by chronic nonsuppurative
cholangitis and granulomata. PBC patients often have concomitant autoimmune diseases, including Sjogren’s
syndrome, Systemic sclerosis, Rheumatoid arthritis and so on. In this review we highlight pathogenesis,
prevalence, diagnosis, treatment and prognosis in primary biliary cirrhosis patients with extrahepatic
autoimmune diseases.