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Basic PSC Facts

PSC does not discriminate based on age or gender.

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.

BASIC FACTS

PSC does not discriminate based on age or gender, but affects more males than 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 progression is different for each person
  • PSC isn’t contagious
  • PSC 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, including:

  • 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.)
  • At this time, 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)
  • Colon cancer, for those who also have IBD (ulcerative colitis or Crohn's disease)

PSC patients sometimes report other symptoms, including: 

  • night sweats, nausea 
  • vitamin deficiency of fat soluble vitamins, A, D, E, or K
  • stool changes 
  • weight loss

In the later stages of PSC: 

  • clotting problems 
  • nail clubbing 
  • jaundice
  • variceal bleeding (spontaneous bleeding in the veins around the esophagus)
  • ascites (fluid in the belly) 
  • hepatic encephalopathy (HE or mental fog), and other problems.

To better understand PSC and related diseases, visit the Medical Terms page on this website.

COMMON PSC MEDICATIONS

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

  • Urso may be used by some physicians for patients diagnosed with PSC. It has produced some anti-inflammatory reactions. Urso helps to reduce toxic bile acids that build up in the liver cells.
  • The benefit of high-dose urso is questionable, and a recent study suggests that high doses may actually be detrimental. For this reason, most hepatologists prescribing urso today will use doses in the range of 15 to 20 mg/kg a day.

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). Rifampin may cause changes in other medications you are taking and alter the way the medicines 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 should not be taken at the same time. 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.

  • Natural 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 on the Educational Resources page of our website. We are also happy to send copies of our brochures to you, by request.

Answers to frequently asked PSC-related questions are available on this website including:

Complete your profile and join PSC Partners Seeking a Cure in advancing PSC research towards a cure.