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PSC: THE FACTS

PSC: THE FACTS

Primary Sclerosing Cholangitis (PSC) is a rare liver disease that damages the bile ducts inside and outside the liver. With PSC, bile ducts are inflamed, and the inflammation leads to scarring and narrowing of the affected ducts. Eventually, blockages may occur. As the scarring blocks more and more ducts, bile becomes trapped in the liver. This damages the liver and can result in fibrosis and cirrhosis of the liver and liver failure. Patients may eventually require a liver transplant.

PSC does not discriminate based on age or gender, but affects about twice as many males as females. Many PSC patients are asymptomatic at the time of diagnosis. It is estimated that there are more than 30,000 PSC patients in the United States.

PSC often is found in people with inflammatory bowel disease (IBD), most often ulcerative colitis (UC) and sometimes Crohn’s disease. More than 75 percent of PSC patients have ulcerative colitis. PSC also is associated with other autoimmune diseases.

Cause

Although no one knows the causes of PSC, research is underway to better understand the disease, develop more effective treatments, and, eventually, find a cure.

  • PSC may have genetic, autoimmune, and environmental origins.
  • PSC isn’t contagious, and no two PSC patients are alike.
  • It cannot be transmitted through kissing, sexual activity, touching, or blood transfusions.
  • Although there may be a genetic predisposition to PSC, most children of PSCers are healthy and unaffected.

Common Symptoms of PSC

Although many patients report few, if any, symptoms, some of the symptoms of PSC include the following:

  • Pruritus or intense itching: particularly on soles of hands or feet, though it can occur anywhere, including in eyes and mouth.
  • Fatigue: Feeling run down, unable to get enough sleep, flu-like exhaustion that does not go away with sleep.
  • Depression: Feelings of despair or hopelessness.
  • Pain: Felt in the right side or in the middle of the abdomen towards the rib cage. This is often termed right upper-quadrant (RUQ) pain. The pain may extend to the shoulder blade area, may be of any intensity, and may last for an indefinite period of time.
  • Jaundice: Yellowing of eyes and skin caused by excess bilirubin that the liver cannot process. May be accompanied by dark urine.
  • Chills and Fever: These may be signs of a bacterial infection in the bile ducts, a condition called cholangitis. A cholangitis attack requires immediate medical attention and antibiotics.

Treatments

There are currently no proven treatments that change PSC’s course or slow its progression, but there are medications and procedures that help relieve some PSC symptoms.

  • An inserted stent can widen a blocked bile duct.
  • Some medications help with the itching that commonly occurs in PSC patients. (See more about medications at the end of this section.)
  • The only definitive treatment is a liver transplant. Some PSCers live long and productive lives with the disease and never need a transplant. Other PSCers end up needing a transplant, and they return to health. In some cases, the PSC returns post-transplant. There are some PSCers who have undergone multiple liver transplants.

Complications

Complications of PSC can include:

  • Bacterial Cholangitis
  • Biliary Stone Disease
  • Dominant Stricture (especially in the common bile duct)
  • Cholangiocarcinoma (cancer of the bile ducts)
  • PSC patients sometimes report other symptoms, including variceal bleeding (spontaneous bleeding in the veins around the esophagus), ascites (fluid in the belly), hepatic encephalopathy (HE or mental fog), night sweats, nausea, vitamin deficiency, stool changes, weight loss, clotting problems, nail clubbing, jaundice, and other problems.

Common PSC Medications

Ursodeoxycholic acid (Ursodiol, Urso Forte, UDCA, URSO, Actigall, Ursofalk)

Urso is a popular medication used for patients diagnosed with PSC. In addition, some anti-inflammatory properties have been noticed. Urso helps to fight toxic bile acids that build up in the liver cells. In the United States, Urso is available under different trade names such as Actigall (typically 300 mg. capsules) and URSO (typically 250 mg tablets). Urso Forte is available in a 500 mg tablet and, along with Urso 250, is the only FDA approved treatment for patients with PBC (Primary Biliary Cholangitis), a disease similar to PSC. Another variation of Urso is available in Australia under the name Ursofalk (250 mg capsules). Ursofalk is also available in a liquid formulation for pediatric patients.

Ursodiol is typically prescribed in typical dosages of 15-30 mg/kg a day. The benefit of high-dose ursodiol is questionable, and a recent study suggests that high doses may actually be detrimental. For this reason, most hepatologists prescribing ursodiol today will uses doses in the range of 15 to 20 mg/kg a day. Another unproven, but suggested benefit of ursodiol may be reduction in risk of colon cancer and cholangiocarcinoma.

Rifampin (Rifampicin)

Rifampin is an antibiotic mostly used for the treatment of tuberculosis. However, because it activates receptors in the gut and liver, it has also been shown to help relieve pruritus (itching). Urso and Rifampin generally work well together. However, Rifampin may cause changes in other medications you are taking and alter the way they react in your system. Make sure your doctor is aware of all other medications you are taking along with Rifampin, in order to avoid any possible conflicts.

Cholestyramine (Questran)

Cholestyramine is used to treat high cholesterol and also has been proven effective in combating pruritus (itching) due to bile blockages. Cholestyramine is not absorbed in the bloodstream; it remains inside the gut until excretion. Cholestyramine and Urso cannot be taken together. The general recommendation is to stagger doses of the two medications at least four hours apart.

Non-Prescription Remedies

PSC Partners Seeking a Cure believes that medical professionals should be the primary source of information for treating your PSC. Check with your doctor before making changes to your health routine or taking any alternative or herbal products.

It is important to remember that a product labeled as “natural” does not necessarily mean it is safe for the PSC patient. Supplements and herbs, despite being “natural,” can be toxic to the liver. The production and distribution of these supplements are not regulated as carefully as the production of prescription medications. In the U.S., the Food and Drug Administration does not test or regulate these products. “Natural” products can be sold with little testing and with no proof of efficacy. Sometimes the herb or supplement itself can cause liver damage. In other cases, impurities or toxins introduced during the preparation of the product may be toxic to the liver.

Other Resources

You can find more information on this website and by downloading these brochures: Living with PSC, Diagnosing PSC, PSC Medications, and Pediatric PSC.

Answers to frequently asked PSC-related questions are available here: www.psc-literature.org/FAQ.htm.