Primary biliary cirrhosis (PBC) is a disease of chronic progressive non-suppurative destruction of intrahepatic bile ducts, eventually leading to liver fibrosis and cirrhosis. The cause of PBC is related to environmental factors, genetic factors, and autoimmune dysfunction, and it occurs in middle-aged women. 90% of patients are women between the ages of 40-55. The onset of PBC is relatively insidious, and there are often no specific symptoms, so the onset of the disease is often not noticed by patients. Main symptoms: weakness, pruritus jaundice, dyspepsia, steatorrhea and metabolic bone disease, skin yellow tumor, hepatosplenomegaly, portal hypertension and esophageal varices, etc. Laboratory tests: 1. Biochemical tests: transaminases are mildly increased or may be normal, early bilirubin is often not significantly increased, and alkaline phosphatase is often significantly increased in almost all patients with PBC, suggesting the presence of intrahepatic cholestasis and damage to small bile ducts. Serum r-glutamyl transpeptidase and cholesterol levels are also elevated. Serum albumin levels are mostly in the normal range, and globulin levels are not significantly increased. 2. Immunological examination: IgG is significantly increased in about 70-80% of PBC patients, IgA and IgM are normal or mildly increased, and serum complement C3 may be decreased. The detection rate of AMA (anti-mitochondrial antibody) is as high as 90% in patients with PBC, among which the M2 subtype is the most specific, which is important for the diagnosis of this disease, especially for asymptomatic PBC patients. It also detects other autoantibodies such as anti-nuclear antibody, anti-smooth muscle antibody, anti-thyroid antibody, etc. Clinical manifestations and diagnosis; Since the disease starts slowly and has a long course, it can be divided into asymptomatic and symptomatic stages (early stage, jaundice free stage, jaundice stage and late stage). PBC can also be accompanied by other autoimmune diseases, including scleroderma, dry syndrome, rheumatoid arthritis, systemic lupus erythematosus, polymyositis, mixed connective tissue disease, thyroiditis, autoimmune thrombocytopenia, anemia and other blood abnormalities, and can also be complicated by interstitial lung fibrosis or interstitial giant cell granuloma. PBC patients may be combined with IgA nephropathy. the clinical presentation of PBC patients varies and lacks specific symptoms and features, so it is difficult to confirm the diagnosis in patients without symptoms, jaundice and cirrhosis. for such patients with relatively early course, if they are middle-aged women, the possibility of this disease should be thought of when they complain of weakness, loss of appetite and mild pruritus of the skin. Laboratory tests must be performed, and if alkaline phosphatase, r-glutamyl transpeptidase and immunoglobulin IgG are increased, AMA (anti-mitochondrial antibody) and other autoantibodies should be detected, and liver tissue should be taken by liver puncture for pathological examination if necessary. Treatment: The principle of treatment for PBC is early active treatment, if the disease is already advanced, the treatment effect is often poor. 1. Symptomatic treatment: pay attention to rest, increase nutrition, supplement fat-soluble vitamins A, D, E and K. While using vitamin D and calcium supplements, increase outdoor activities appropriately when physical strength permits, in order to reduce bone thinning and osteoporosis. 2, symptomatic treatment: (1) skin itching: bile stagnation is one of the causes of skin itching, caused by the accumulation of bile salts in the skin, the commonly used drugs clinically are bilirubin 12g/d, divided into 3 oral doses, but the adverse effects of bilirubin include nausea and epigastric discomfort, this drug can be combined with thyroxine, digoxin, oral contraceptives, ursodeoxycholic acid and other drugs, so do not take with such drugs. If oral ursodeoxycholic acid is required, the two drugs should be taken at least 4 hours apart. (2) Osteoporosis: Encourage patients to exercise properly, get more sunlight, take oral calcium tablets 1-1.5g per day, take oral or intramuscular vitamin D 500-5000 units, the specific dose depends on the patient’s condition, also take oral alfa osteoporosis 0.25-0.5ug once a day, and take oral sodium allantoin phosphate 70mg/week. 3. Ursodeoxycholic acid 10-15mg/(kg.d) body weight, divided into 3 oral doses per day, it is best to use this drug in the early stage of the disease, and the course of treatment should be long, wait until PBC enters the late stage, the efficacy is often not significant. Ursodeoxycholic acid should be used simultaneously with immunosuppressants or other drugs, which is more beneficial to control the disease. Colchicine is a drug for the treatment of gout, because it has the function of reducing collagen synthesis, enhancing the activity of collagenase and regulating the function of cytokines, and also has the function of anti-fibrosis, so it is also used in the treatment of PBC. The main side effects of this drug can cause diarrhea, loss of appetite, nausea, upper abdominal discomfort and other gastrointestinal reactions, and a few patients may have granulocytopenia, and the dose should be reduced or discontinued if the adverse reactions are obvious. 5, penicillamine has a strong complexation of copper, lead, mercury and other metal ions, repel the accumulation of these metal ions in the body, and inhibit the inflammatory response, prevent collagen formation and reduce PBC patients. D-penicillamine is commonly used clinically, starting at a dose of 125mg orally and increasing by 125mg every 2 weeks until it is maintained at 500mg daily. Common side effects include gastrointestinal discomfort, skin rash, neutropenia and thrombocytopenia, proteinuria and hematuria, etc. Changes in blood phase and renal function should be observed during the course of dosing. 6. Cyclosporine A has the function of inhibiting the production of cytokines and lymphocytes, thus regulating the immune response of the body. 2-4mg/(kg.d), generally adults take 200mg orally every day, because cyclosporine A has toxic effect on the kidney, the kidney function must be monitored during the course of medication, and the course of treatment should not be too long. 7. Methotrexate 10-15mg once a week. Changes in blood and liver function should be noted during the drug administration. Interstitial pneumonia may occur in some patients. Patients may develop oral ulcers while taking methotrexate, which can be prevented by taking oral folic acid. 8. Adrenocorticosteroids: PBC patients treated with oral prednisolone or methylprednisolone can reduce skin itching and fatigue symptoms, and serum transaminase and alkaline phosphatase are also reduced to some extent. However, after long-term use of glucocorticoids, it can promote osteoporosis and bone thinning of patients and aggravate metabolic bone disease, so many scholars do not recommend PBC patients to receive long-term treatment with adrenal hormones. 9, liver transplantation: PBC is one of the indications for liver transplantation treatment. It is believed that patients with PBC should consider liver transplantation when their serum bilirubin exceeds 145 mmol/L or when their liver function is decompensated, and the literature reports that the one-year survival rate after liver transplantation is 75% and the five-year survival rate is 70%, which is better than that of patients who did not receive liver transplantation. The outcome of liver transplantation is related to the patient’s condition at the time of treatment; if the disease is very advanced and already quite severe, the outcome after transplantation is often unsatisfactory. After transplantation, patients need to take oral cyclosporine A and other immunosuppressive drugs for a long time to prevent immune rejection. V. Prognosis: The prognosis of asymptomatic PBC is better than that of symptomatic patients. However, PBC is a progressive disease, and its clinical manifestations gradually worsen with the prolongation of the disease course. It is difficult to determine how long it takes for asymptomatic patients to transition to the symptomatic stage. The literature reports that 27-89% of asymptomatic patients develop clinical symptoms after 27-89 months. For a specific patient, the prognosis is significantly related to the patient’s state of mind, financial status, and the appropriateness of treatment measures, in addition to the early stage of the disease and the severity of the disease.