What is biliary cirrhosis and how is it caused? Biliary cirrhosis often refers to cirrhosis caused by primary sclerosing cholangitis, an inflammatory disease in which the bile ducts are narrowed or occluded due to chronic fibrosis of the intrahepatic and extrahepatic bile ducts and thickening of the walls of the bile ducts, which is also known as stenosing cholangitis. This disease is clinically rare, due to the progressive aggravation of the patient’s condition leads to intrahepatic fibrous tissue proliferation, portal vein pressure increases, the formation of cirrhosis combined with portal hypertension, and ultimately because of cholestasis-induced liver failure and death, the disease is currently the lack of effective treatments, the patient’s prognosis is extremely poor. Biliary cirrhosis is closely related to chronic inflammatory bowel disease, autoimmune disease and genetic factors. The disease can involve all parts of the intrahepatic and extrahepatic bile ducts. The most common form of the disease is a marked thickening of the wall of the extrahepatic bile ducts and narrowing of the lumen. The bile duct wall is fibrotic and the lumen is narrowed. Histology is characterized by inflammatory cell infiltration and fibrosis of the bile duct mucosa. Patients with biliary cirrhosis have a significantly higher risk of developing cholangiocarcinoma. There is a lack of effective treatment for this disease, ursodeoxycholic acid can be given to reduce the level of bilirubin and aminotransferase, but it can’t improve the clinical symptoms and delay the course of the disease, and antibiotics can be given to the patients when they are combined with cholangitis. Liver transplantation is the last resort for end-stage treatment of biliary cirrhosis, with a 5-year survival rate of 85%.