What do you know about primary biliary cirrhosis?

Primary biliary cirrhosis is a chronic progressive autoimmune disease that occurs predominantly in female patients older than 40 years of age and less frequently in those younger than 25 years of age. Histologic manifestations are characterized by inflammation of the confluent area and immune-mediated destruction of small intrahepatic bile ducts. Immunologic abnormalities are characterized by the presence of three specific autoantibodies, ANA, SMA, and core protein gp210, core protein p62, and nuclear fibrillar layer protein sp100, in addition to positive anti-mitochondrial antibodies. The pathogenesis of primary biliary cirrhosis is still unclear, ursodeoxycholic acid (Yusuf) is the only drug approved by the FDA for the treatment of patients with primary biliary cirrhosis, and there is basically no treatment other than Yusuf in Western medicine, and the use of Yusuf alone is ineffective in patients with advanced stage, which makes many patients face the situation of having no medicine to save them. For patients with advanced PBC, liver transplantation is the only treatment option. The 1-year survival rate after liver transplantation is 92%, and the 5-year survival rate is 85%. However, the recurrence rate is 15% at 3 years and 30% at 10 years after surgery. Primary biliary cirrhosis is an autoimmune liver disease, a chronic intrahepatic cholestasis of unknown cause, culminating in cirrhosis and liver failure. Clinical manifestations include fatigue and weakness, generalized itching, jaundice, hyperpigmentation and/or yellow tumors. Abdominal pain, nausea, vomiting, edema, ascites and bleeding from ruptured esophageal varices can also be the first manifestation. Primary biliary cirrhosis occurs more often in women above middle age, and the incidence of women accounts for about 80-90%. Clinical manifestations: 1, itching of the skin, jaundice, dark yellow urine, lighter feces, skin pigmentation; 2, fatigue, abdominal pain, nausea, vomiting, loss of appetite, weight loss; 3, steatorrhea, skin roughness, night blindness, bone softening, osteoporosis, hemorrhagic tendency; 4, xanthomas; 5, hepatosplenomegaly, spider nevus, and anemia; 6, advanced cutaneous and mucosal hemorrhage, ascites, rupture of esophageal varices and hemorrhage and hepatic encephalopathy; 7, hepatocerebral hemorrhage, and hepatic cerebral disease. Hemorrhage and hepatic encephalopathy; 7, can be accompanied by dry syndrome, scleroderma, calcification – Raynaud’s disease – dermatofinger (toe) sclerosis and capillary syndrome, the corresponding manifestations of chronic thyroiditis. Diagnosis based on: 1, middle-aged women; 2, obvious pruritus, jaundice, yellow tumor, hepatosplenomegaly; 3, elevated serum ALP, r-GT, etc.; 4, elevated serum bilirubin bile acids; 5, positive serum mitochondrial antibodies, elevated IgM, positive antinuclear antibodies, anti-DNA antibodies, rheumatoid factor, anti-thyroid antibodies, etc.; 6, liver biopsy Pathologic examination can confirm the diagnosis.