Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease of undetermined cause. The pathology is characterized by non-suppurative inflammation of the intrahepatic bile ducts, accompanied by bile duct destruction, periportal inflammation and fragmented necrosis of the liver parenchyma, eventually progressing to cirrhosis. The disease has an insidious onset and the main clinical manifestations are: 1. malaise: the most common complaint, which can appear early in the disease. 2. pruritus: another common clinical manifestation, varying in severity. It may be related to the deposition of bile acids in the skin.3. Jaundice: obstructive jaundice is one of the important clinical manifestations of PBC, suggesting significant damage to the intrahepatic bile duct. Jaundice often appears several months or years after the onset of the disease. The deeper the jaundice or the faster the rate of jaundice deepening, the more serious the disease. However, many patients do not have jaundice.4, dyspepsia: such as abdominal distension, poor appetite, belching, etc.5, steatorrhea and metabolic bone disease: steatorrhea is a manifestation of PBC later in the course of the disease, often accompanied by persistent and obvious jaundice. Long-term steatorrhea will lead to deficiency of fat-soluble vitamins. Vitamin A malabsorption can cause visual impairment. Malabsorption of vitamin D may lead to metabolic bone disease, in which osteoporosis is more common and may be complicated by osteochondrosis, manifested by bone pain and pathological fractures.6. Yellow tumor of the skin: associated with hypercholesterolemia in PBC patients.7. Hepatosplenomegaly.8. Portal hypertension and esophageal varices: various manifestations and complications of cirrhosis will appear in the late stage of the disease. The clinical stage can be divided into 4 stages: asymptomatic stage with normal liver function, abnormal liver function, symptomatic stage, and decompensated stage. early laboratory findings of PBC show features of cholestasis, with elevated serum bilirubin, accompanied by significantly increased alkaline phosphatase and γ-glutamyl transaminase, suggesting intrahepatic cholestasis and small bile duct injury. However, early in the course of the disease, serum bilirubin levels are often not significantly elevated. Serum aminotransferases may be mildly elevated or normal. Serum cholesterol and lipoproteins are often elevated. Serum albumin is normal early in the disease, globulin is often elevated, especially serum IgM is characteristically elevated, and IgA and IgG are normal or mildly elevated. Anti-mitochondrial antibodies are very important for the diagnosis of PBC, and the detection rate can be over 90%, among which the M2 subtype is the most specific, which is important for the diagnosis of the disease, especially for asymptomatic PBC patients. other autoantibodies, such as anti-nuclear antibodies, anti-smooth muscle antibodies and anti-thyroid antibodies, can also be detected in the serum of PBC patients. liver pathological changes of PBC It can be divided into 4 stages: Stage 1:The stage of cholangitis, mainly chronic non-suppurative inflammation of the interlobular bile ducts or septal bile ducts of the liver, with lymphocytes, plasma cells, histiocytes and a few eosinophils infiltrating the lumen, wall and surroundings of the bile ducts, while enlarging the confluent area, with lymphocytes and typical granuloma formation in the confluent area in about half of cases. There is little destruction of hepatocytes and hepatocyte boundary plates, and the hepatic lobules are structurally intact without bile retention. Stage 2:It is the stage of fine bile duct hyperplasia, characterized by hyperplasia of small bile ducts and disappearance of interlobular bile ducts, which are surrounded by inflammatory cells or fibroblasts, hence the name peribiliary cholangitis. The hepatocytes are mostly normal, but there is siltation of the hepatocytes around the inflamed confluent area. The perifollicular capillary bile duct lumen is enlarged, microvilli are absent, shortened or edematous, and mitochondria are enlarged. Stage 3: scarring stage, inflammation decreases, leaving stellate scarring, and scar tissue in the confluent area extends into another confluent area or into the hepatic lobules. Cholestasis becomes more severe. Stage 4: In cirrhosis, the hepatocytes are focally necrotic and the fibrous septa in the confluent area expand and join each other, separating the lobules and forming pseudobullets and regenerative nodules. When the pseudobullets form, they distort the blood vessels.