Symptoms and Signs of Primary Sclerosing Cholangitis

Medical Author:
Medically Reviewed on 3/4/2022

Doctor's Notes on Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), rare, progressive disease of the bile ducts (inflamed, scarred, and thickened) that occurs more often in men (70%). The signs and symptoms are slowly progressive, so early in the disease, most people will not have any symptoms. Often, the disease can produce elevated blood levels of liver enzymes that are incidentally found. Jaundice, dark urine, and pruritus can develop as obstruction of liver bile ducts increase; abdominal pain, fever, and fatigue also increase.

Although about 10% of patients are known to have an autoimmune hepatitis form of the disease that advances more rapidly; however, the cause of primary sclerosing cholangitis is not known. It has been associated with Crohn's disease and ulcerative colitis.

What Are the Treatments for Primary Sclerosing Cholangitis (PSC)?

Treatments for PSC center mainly on symptoms and complications of the disease. The chief symptom is pruritus (itching), and the major complications are infections; some compounds do both:

  • Treatments for infections
    • Rifampin also reduces itch
    • May use repeated doses of various antibiotics due to blocked bile ducts and as a preventative of infection if any invasive procedure is done
    • Bile acid sequestrants bind to bile acids and reduce itching.
    • Ursodeoxycholic acid increases bile absorption.
    • Opioid antagonists reduce itch sensation in the brain.
  • Nutrition: may need vitamin supplements; oral or IV calcium and vitamin D
  • Bile duct blockage treatments
    • Balloon dilation of the larger bile ducts
    • Stent placement to keep the bile duct open
  • Liver transplant: removal of PSC liver and replaced with a compatible normal liver may cure PSC

Your doctors can determine what treatments your PSC warrants.

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REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.