Also known as stenosing cholangitis, it is not essentially a suppurative disease. It is a disease of unknown cause, characterized by chronic fibrotic stenosis and occlusion of the intrahepatic and extrahepatic bile ducts. It is relatively rare clinically. It differs from sclerosing cholangitis (or secondary biliary stricture) caused by bile duct stones, tumors, or post-injury bile duct damage. Primary sclerosing cholangitis generally does not involve gallstones or a history of biliary surgery, and many cases are accompanied by ulcerative colitis. A small number of patients also have conditions such as fibrous thyroiditis and retroperitoneal fibrosis. The age of onset is mostly between 30 and 50 years, with males being more affected than females. Currently, bacterial and viral infections, immune dysfunction, and certain congenital genetic factors are considered potential disease-causing factors.

bubble_chart Pathological Changes

80% of the lesions involve the entire biliary system, including the gallbladder, while 20% are confined to the extrahepatic biliary system. The hepatic duct confluence is generally the most severely affected. The external diameter of the affected extrahepatic bile ducts shows little change, but due to the thickening of the duct walls, the lumen becomes significantly narrowed, with an internal diameter that may be less than 2mm. However, the bile within the ducts mostly remains clear. Similar changes can occur in the intrahepatic bile ducts, and in the late stage [third stage], biliary cirrhosis and portal hypertension may develop.

bubble_chart Clinical Manifestations

It is mainly obstructive jaundice, presenting as a slow, progressive process. Typically, there is no history of colicky pain in the upper abdomen, only discomfort and dull pain, accompanied by significant skin itching, loss of appetite, nausea, and lack of strength. A few patients may experience fear of cold and fever. Blood tests show increased lymphocytes and eosinophils, elevated serum bilirubin, alkaline phosphatase, and γ-glutamyl transpeptidase levels, grade I elevation of alanine aminotransferase, and IgM levels above normal. Some patients test positive for antinuclear antibodies and smooth muscle antibodies, while anti-mitochondrial antibodies are negative. Copper levels in the liver and urine are elevated.

Previously, the diagnosis was only confirmed when a hard, cord-like change in the bile duct was observed during surgical exploration. Intraoperative cholangiography revealed diffuse and irregular narrowing of the bile ducts, and a biopsy of the bile duct wall ruled out the presence of cholangiocarcinoma. Combined with the manifestation of progressive obstructive jaundice, the absence of gallstones, and no history of bile duct surgery, a diagnosis of primary sclerosing cholangitis could be made. However, the final diagnosis must still be confirmed after at least five years of follow-up, provided no malignant tumors of the bile duct develop. Due to the difficulty in differentiating between cholangiocarcinoma and primary sclerosing cholangitis, even if a biopsy shows no cancer, it is sometimes challenging to completely exclude cholangiocarcinoma. In recent years, the widespread use of ERCP and PTC has made it possible to diagnose the condition preoperatively. In patients with obstructive jaundice disease, especially those with ulcerative colitis, the possibility of this disease should be considered. ERCP examination can reveal diffuse, bead-like, and band-like narrowing changes in the intrahepatic and extrahepatic bile ducts.

bubble_chart Treatment Measures

Currently, there is still a lack of specific treatment methods. The goal of surgical treatment is to drain the gallbladder and decompress the bile ducts to reduce liver damage. During surgical exploration of the biliary tract, biopsies of the bile duct wall and liver should be performed, along with intraoperative cholangiography and aerobic and anaerobic bacterial cultures of the gallbladder. For localized strictures, dilation of the narrowed area can be performed, followed by the placement of a T-tube, catheter, or plastic tube for drainage. The drainage tube can be inserted through the bile duct incision or the liver surface. It is recommended to leave the drainage tube in place for at least one year, and some advocate for long-term or even lifelong retention. Sometimes, the lumen of the extrahepatic bile duct is too narrow, making tube drainage difficult or unsuccessful. Although some suggest performing biliary-enteric internal drainage for strictured bile ducts, this method is rarely adopted due to technical difficulties and suboptimal decompression effects. Generally, if a patient improves with medical treatment or has developed gallbladder-induced cirrhosis, surgical intervention is not recommended. Some believe that if the patient also has active ulcerative colitis, a colectomy may help stabilize the condition of primary sclerosing cholangitis. In recent years, some have attempted catheter insertion and bile duct balloon dilation via endoscopy or percutaneous liver puncture, but the success rate is not high. Medical treatment primarily involves long-term use of corticosteroid drugs, which can alleviate symptoms but generally do not alter the disease course. Early-stage results are relatively better, while late-stage [third-stage] outcomes are less satisfactory. Broad-spectrum antibiotics can control acute inflammation of the bile ducts and may be used in combination with steroids. Some also use immunosuppressants such as azathioprine, but the efficacy remains uncertain.

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bubble_chart Prognosis

The prognosis of primary sclerosing cholangitis is poor. It is believed that this condition will eventually develop into cholangiocarcinoma. The final outcome is mostly secondary biliary cirrhosis and portal hypertension. Most patients die from liver failure or hepatic coma, but death due to rupture and massive bleeding of esophageal varices is rare. Most patients can only survive for 5 to 10 years after diagnosis, with an average of 6 to 7 years. Those with concurrent ulcerative colitis have an even worse prognosis.