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Primary sclerosing cholangitis.

 
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Last reviewed: 12.07.2025
 
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Primary sclerosing cholangitis is a chronic cholestatic syndrome characterized by patchy inflammation, fibrosis, and intra- and extrahepatic strictures of the biliary tract. Eighty percent of patients have inflammatory bowel disease, most commonly ulcerative colitis. Symptoms of fatigue and pruritus develop late. Diagnosis is based on contrast cholangiography (ERCP) or magnetic resonance cholangiopancreatography. The disease ultimately leads to obliteration of the bile ducts with the development of cirrhosis, liver failure, and sometimes cholangiocarcinoma. If the disease progresses, liver transplantation is indicated.

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What causes primary sclerosing cholangitis?

The cause is unknown. However, primary sclerosing cholangitis (PSC) is closely associated with inflammatory bowel disease; approximately 5% of patients have ulcerative colitis and approximately 1% have Crohn's disease. This association and the presence of certain autoantibodies [eg, anti-smooth muscle and perinuclear antineutrophil antibodies (pANCA)] suggest immune-mediated mechanisms. T cells appear to be involved in bile duct injury, suggesting a cellular immune disorder. A genetic predisposition is suggested by a family history and a higher incidence among individuals with HLA B8 and HLA DR3, which are often associated with autoimmune diseases. Unproven triggers (eg, bacterial infection or ischemic duct injury) probably trigger the development of primary sclerosing cholangitis in genetically susceptible individuals. Sclerosing cholangitis in HIV-infected patients may be cryptogenic or caused by cytomegalovirus.

Causes of Primary Sclerosing Cholangitis

Symptoms of Primary Sclerosing Cholangitis

The average age at diagnosis is 40 years; 70% of patients are men. The onset of the disease is usually gradual, without obvious clinical symptoms, with progressive malaise and pruritus. Jaundice usually develops later. Recurrent episodes of right-sided abdominal pain and fever, possibly due to ascending bacterial infection of the biliary tract, occur in 10-15% of patients at clinical presentation, with right-sided pain typical. Steatorrhea and signs of fat-soluble vitamin deficiency may be present. Persistent precursors of jaundice characterize the progression of the disease. Clinically manifest gallstones and choledocholithiasis usually develop in about one third of patients. In some patients, the disease is asymptomatic for a long time, initially manifesting as hepatomegaly or cirrhosis. The terminal phase of the disease includes decompensated liver cirrhosis, portal hypertension, ascites and liver failure.

Despite the association of primary sclerosing cholangitis with inflammatory bowel disease, the two diseases usually occur separately. Ulcerative colitis may manifest several years before primary sclerosing cholangitis, but it is usually associated with a milder course of primary sclerosing cholangitis. The presence of both diseases increases the risk of colorectal cancer, regardless of whether liver transplantation has been performed for primary sclerosing cholangitis. Similarly, total colectomy does not alter the course of primary sclerosing cholangitis. Cholangiocarcinoma develops in 10-15% of patients with primary sclerosing cholangitis.

Symptoms of Primary Sclerosing Cholangitis

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Diagnosis of primary sclerosing cholangitis

Primary sclerosing cholangitis is suspected in patients with unexplained abnormal liver function tests; if the patient has inflammatory bowel disease, the suspicion of primary sclerosing cholangitis is heightened. Biochemical abnormalities of hepatic cholestasis are typical, with alkaline phosphatase and gamma-glutamyl transferase levels usually being more elevated than aminotransferase levels. IgG and IgM levels are usually elevated, and anti-smooth muscle antibodies and pANCA tests are usually positive. Antimitochondrial antibodies, which are positive in primary biliary cirrhosis, are negative.

The hepatobiliary evaluation usually begins with ultrasound to exclude extrahepatic biliary obstruction. The diagnosis of primary sclerosing cholangitis requires identification of multiple strictures and dilations involving the intra- and extrahepatic bile ducts - this is done by cholangiography (ultrasound can only suggest their presence). Direct cholangiography (eg, ERCP) is the "gold standard"; however, magnetic resonance cholangiopancreatography (MRCP) provides better images and is becoming the main alternative noninvasive diagnostic method. Liver biopsy is usually not required to verify the diagnosis. If biopsy is performed for other indications, it demonstrates bile duct proliferation, periductal fibrosis, inflammation and obliteration of the bile ducts. As the disease progresses, fibrosis spreads from the portal areas and ultimately leads to biliary cirrhosis of the liver.

Dynamic examination using ERCP and a cytology biopsy brush will help in predicting the development of cholangiocarcinoma.

Diagnosis of primary sclerosing cholangitis

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Treatment of primary sclerosing cholangitis

In some patients, the disease may be asymptomatic for several years despite progressive changes. The time interval from diagnosis verification to the development of liver failure may be about 12 years.

Asymptomatic course generally requires only observation and monitoring (e.g. physical examination and liver function tests twice a year). Ursodeoxycholic acid can reduce skin itching and improve biochemical markers. Chronic cholestasis and liver cirrhosis require treatment. In case of recurrent bacterial cholangitis, antibacterial therapy and ERCP are performed as indicated.

Treatment of primary sclerosing cholangitis

Prognosis of primary sclerosing cholangitis

If a dominant stricture is detected (approximately 20%), endoscopic dilation is necessary to relieve symptoms and cytology to exclude a tumor. Any infection (eg, cryptosporidiosis, cytomegalovirus) should be treated.

Liver transplantation, the only treatment that increases survival in idiopathic primary sclerosing cholangitis, can lead to a cure. Recurrent bacterial cholangitis or complications of end-stage liver disease such as severe ascites, portosystemic encephalopathy, bleeding from esophageal varices are indications for liver transplantation.

Prognosis of primary sclerosing cholangitis

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