Primary biliary cirrhosis (PBC) is a disease of unknown origin characterized by progressive destruction of intrahepatic bile ducts and chronic cholestasis. Pathogenesis: Primary biliary cirrhosis undergoes four typical stages of development. Stage I involves erosion of normal bile ducts with lamellar inflammation and destruction of interstitial and interlobular bile ducts, and granulomas may also be found. Stage II is followed by bile duct hyperplasia and destruction of the portal duct area, with the spread of inflammation to the liver parenchyma, massive bile duct hyperplasia, and periportal fibrosis formation. In stage III, bile duct hyperplasia and inflammation decreased, and fibrous cords were attached to the portal area. In stage IV, hard, regular, regenerative nodules with obvious bile staining appear, forming cirrhosis. In the absence of granulomas and characteristic bile duct lesions it is difficult to distinguish primary biliary cirrhosis from other types of cirrhosis. The problem associated with histologic staging is that there is significant overlap between the above-mentioned stages, especially stages II and III, and that the stages may not reflect the clinical status (e.g., a patient with stage III lesions may have no clinical manifestations). Signs and symptoms: Although primary biliary cirrhosis can occur in both sexes in all age groups, it occurs in more than 90% of female patients between the ages of 35 and 70 years and tends to be latent. Approximately 50% of patients are asymptomatic at the time of detection, with only abnormalities on routine serum biochemical tests. In about 50% of patients, pruritus and/or unexplained fatigue are the initial symptoms, and other clinical manifestations may not appear until months or even years later. The liver is enlarged and firm without tenderness in about 50% of patients; the spleen is enlarged in 25% of patients; both cutaneous xanthomas and macular tumors occur in about 15% of patients; hyperpigmentation occurs in 10% of patients; and jaundice develops in 20% of patients and progresses over time in combination with other symptoms. Other possible clinical manifestations include pestle finger, metabolic bone disease (e.g., osteoporosis), peripheral neuropathy, moderate renal tubular acidity, and steatorrhea (due to cholestasis and pancreatic secretion dysfunction). As the disease progresses, all features and complications of cirrhosis may appear. Primary biliary cirrhosis is often associated with autoimmune diseases (e.g. rheumatoid arthritis, scleroderma, desiccation syndrome and autoimmune thyroiditis). Laboratory tests: The early laboratory findings of the disease are characterized by cholestasis and are accompanied by a disproportionate increase in alkaline phosphatase and gamma-glutamyl transaminase with serum bilirubin and transaminase. In fact, serum bilirubin levels are often normal in the early stages of primary biliary cirrhosis. Serum cholesterol and lipoproteins are often elevated, serum albumin is normal early in the disease, globulins are often elevated, especially serum IgM is characteristically elevated, and anti-mitochondrial antibodies against a component of the mitochondrial lining are important for diagnosis (present in >95% of patients), but can also be detected in autoimmune chronic active hepatitis. Diagnosis and prognosis: Differential diagnoses include: extrahepatic biliary obstruction, chronic active hepatitis, primary sclerosing cholangitis, and pharmacologic cholestasis. Potentially curable extrahepatic biliary obstruction should be ruled out as soon as possible. Ultrasonography and, in some cases, ERCP are also necessary. Liver biopsy can confirm the diagnosis, but is often nonspecific. In rare cases, a diagnostic dissection is required. The progression of primary biliary cirrhosis varies, from several years without affecting the patient’s quality of life to 2-7 years after the onset of symptoms in patients without clinical manifestations. Some patients have only mild symptoms for 10-15 years, while others deteriorate in 3-5 years. Increased serum bilirubin with autoimmune disorders and progressive histological changes suggest a poor prognosis. The survival time for bilirubin >6mg/dl (100μmol/L) is <2 years. When pruritus disappears, macular tumor shrinks, and serum cholesterol decreases, the prognosis is good. The final presentation of primary biliary cirrhosis is similar to that of other types of cirrhosis: portal hypertension and esophageal varices, ascites, liver and renal failure. Treatment: No specific treatment is available. Pruritus can be controlled with bilirubin, 6-12 g daily, divided into oral doses. Osteoporosis is particularly difficult to treat, but estrogen may have some effect. Patients with steatorrhea need supplementation with Ca and vitamins A, D and K to prevent their deficiency. The treatment of complications of primary biliary cirrhosis is the same as for other types of cirrhosis. Corticosteroids have been used, but are generally contraindicated because they can aggravate osteoporosis. In many clinical trials, the use of other immunosuppressive agents has also failed to improve the quality of life or extend the life span of patients. Colchicine 0.6mg twice daily may reduce liver fibrosis and may have some effect. Ursodeoxycholic acid 10mg/kg daily can improve liver function, increase survival rate and delay liver transplantation. Primary biliary cirrhosis is one of the best indications for liver transplantation, and its efficacy is better.