Primary biliary cirrhosis is an autoimmune-related progressive cholestatic liver disease, which is mainly characterized by progressive destruction of small intrahepatic bile ducts with inflammatory changes in the portal vein, eventually leading to fibrosis and cirrhosis. It often coexists with other immune diseases such as rheumatoid arthritis, dry syndrome, scleroderma, chronic lymphocytic thyroiditis, etc. It has an insidious onset and passes through slowly, with mild symptoms in the early stage, and the patients are in good general condition, with no obvious decrease in appetite and weight, and about 10% of the patients can be asymptomatic. For patients with chronic progressive obstructive jaundice of unknown cause, especially those with steatorrhea, we should know in detail about the causative factors of the disease and the progress of the disease, whether there are other immune diseases, and pay attention to differentiate from secondary biliary cirrhosis and other causes of jaundice in cirrhosis. Laboratory tests: Lipids, serum bile acids, conjugated bilirubin, alkaline phosphatase, r-glutamyl transpeptidase and other microbiliary enzymes are markedly elevated, and nitrogenase is normal or mildly or moderately elevated. The blood was positive for anti-mitochondrial antibodies with elevated IgM, positive for antinuclear antibodies, positive for urinary bilirubin, and normal or decreased urinary bilirubin. Imaging Ultrasound, ERCP, CT, and PTC are used to understand the presence of intra- and extra-hepatic bile duct dilatation and diseases causing extra-hepatic obstructive jaundice to exclude secondary biliary cirrhosis.