What are the clinical manifestations of biliary cirrhosis? The majority of biliary cirrhosis patients are male, and the age of onset is 40-45 years old. Occasionally, it is seen in children. Patients with biliary cirrhosis often suffer from thickening of the walls of the bile ducts, narrowing of the lumen, and bile stasis leading to hepatocellular damage because the lesions involve all parts of the bile ducts inside and outside the liver. The majority of patients have intermittent episodes, and there may be no obvious symptoms between episodes. The clinical features of the attacks are usually characterized by persistent painless jaundice, itching, fever, malaise, and indifference. In severe cases, cholangitis may be characterized by chills and high fever. Some patients have mild clinical symptoms and often have intermittent attacks. When the patient’s condition develops to the advanced stage, it often manifests intractable ascites, upper gastrointestinal bleeding, black stools, coma and other symptoms. Laboratory tests often show elevated serum total bilirubin, with a significant increase in direct bilirubin, accompanied by elevated alkaline phosphatase, plasma copper, copper cyanin and urinary copper. Plasma IgM levels are increased in about half of the patients. In recent years, biliary magnetic resonance imaging has become the most effective diagnostic method for this disease. It may be characterized by diffuse irregular multiple strictures of the intra- and extrahepatic bile ducts. Stenosis is most severe at the confluence of the right and left hepatic ducts. The bile duct branches are stiff and thin or mildly dilated. The bile ducts may also show annular stenosis with post-stenotic dilatation and bead-like changes. There is still a lack of effective treatment for this disease.