Genetic primary cholangitis

All the diseases we encounter in life are primary. They are found to be caused by congenital factors, and the body itself carries this gene, which leads to the occurrence of the disease. The liver itself has immune ability, but due to many factors, the liver's own immune ability decreases. Is primary cholecystitis hereditary?

Primary sclerosing cholangitis.

Primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune liver disease are autoimmune diseases of the liver. Primary sclerosing cholangitis is a chronic cholestatic syndrome characterized by extensive inflammation and fibrosis of the intrahepatic and extrahepatic biliary system. It mainly affects young people, with an average age of 40 years old, and 70% of patients are male. The disease often develops progressively and eventually develops into biliary cirrhosis and portal hypertension. If liver transplantation is not performed, patients often die of liver failure. Primary sclerosing cholangitis is often accompanied by inflammatory bowel disease, especially chronic ulcerative colitis. Similar to primary biliary cirrhosis, a range of cholestatic complications, such as pruritus, osteoporosis, deficiencies of fat-soluble vitamins, and hypercholesterolemia and manifestations of advanced liver disease, may also occur in primary sclerosing cholangitis. In addition, primary sclerosing cholangitis has some specific complications, such as cholangitis, bile duct stricture, cholelithiasis, cholangiocarcinoma, and varices in the stoma after colorectal resection and ileostomy for chronic ulcerative colitis. Cholangiocarcinoma occurs in about 10% of patients.

The clinical manifestations of primary sclerosing cholangitis can be divided into asymptomatic but abnormal liver function, or due to chronic cholestasis, recurrent cholangitis, complications of chronic liver disease, or accidental discovery during laparotomy.

The most common symptoms of primary sclerosing cholangitis are fatigue, itching, and jaundice. There may also be weight loss, fever and other discomforts. Physical signs may include hepatomegaly, jaundice, splenomegaly, hyperpigmentation, xanthomas, etc. Some patients have complications of inflammatory bowel disease, especially ulcerative colitis, and have corresponding intestinal manifestations.

The laboratory tests of this disease mainly show elevated serum alkaline phosphatase, serum transaminase and serum bilirubin.

Imaging examination of this disease: Cholangiography shows diffuse, multifocal annular stenosis, short-band stenosis, and diverticular protrusions in the intrahepatic and/or extrahepatic bile ducts.

Treatment of this disease: There is currently no specific medicine or effective treatment for this disease. Treatment can be done by balloon dilatation, bile duct reconstruction, liver transplantation and proctectocele. Medical treatment mainly uses some immunosuppressants, anti-fibrosis drugs, and choleretic drugs. Liver transplantation is the only treatment measure that can save the lives of patients with end-stage primary sclerosing cholangitis. Generally speaking, liver transplantation should be considered early before the occurrence of bile duct cancer and advanced liver failure, because most patients do better after liver transplantation, and the five-year survival rate can reach 75% to 85%.