Basic Facts About PSC

PSC, primary sclerosing cholangitis, causes the bile ducts inside and outside the liver to become scarred, narrowed and eventually blocked. As more and more ducts are blocked, bile becomes trapped and damages the liver. The damage, if left unchecked, causes liver cell death, which leads to cirrhosis and may eventually require a liver transplant.

PSC is a rare disease that predominantly affects males ages 30-40 years old. About 43 percent do not have any symptoms when they are diagnosed. It is estimated that nearly 21 per 100,000 men and 6 per 100,000 women have the disease. PSC is often found in people with inflammatory bowel disease (IBD), most often ulcerative colitis (UC) and sometimes Crohn's disease. Over 75 percent of PSC patients have ulcerative colitis. PSC is also associated with other autoimmune diseases. Click here to learn more about autoimmune diseases related to PSC.

Cause: No one knows what causes PSC. There may be genetic, autoimmune, and environmental origins. It isn't contagious. PSC can't be transmitted, through kissing, sexual activity, touching, or blood transfusion. There may be a genetic predisposition to PSC; however, most children of PSCers are healthy and unaffected.

There are medications and procedures that can help relieve some aspects of the disease and symptoms, but the only definitive treatment is a liver transplant. Still, many PSCers can live long and productive lives with the disease, and may never need a transplant.

You can find more information on this website. Download our brochures, which you may find useful to explain the disease to physicians and family. Our brochures, pdf-iconLiving with PSC, pdf-iconDiagnosing PSC, pdf-iconPSC Medications, and pdf-iconPediatric PSC are available by clicking here.

General PSC Frequently Asked Questions are available by clicking here.

Complications of PSC can include bacterial cholangitis, biliary stone disease, dominant stricture (especially in the common bile duct), and cholangiocarcinoma (cancer of the bile ducts). In advanced PSC, patients may experience hepatic encephalopathy, varices, ascites, bleeding disorders, 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 (primary sclerosing cholangitis). Originally prescribed for the treatment of gallstones, Urso has been shown to help bile acids travel through the body with greater ease. 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. Treatment tested over the years has worked for many patients. Always ask your doctor when you make any change in your health routine and what you are doing to manage your disease.

When you don't feel well, when you're frightened by PSC's unclear outcome, it's easy to think that trying some remedies the doctor may not have mentioned is the thing to do. Some people figure they have nothing to lose; they'll try anything.

We believe it is prudent to be cautious with over the counter remedies, vitamins/minerals, and herbals. PSC and its related diseases are complicated and we laymen just don't know enough-it could even be unsafe to try some things. For example, because PSC is an autoimmune disease in which your immune system is highly active, you will want to stay away from immune-boosting products, which could make your condition worse.

Be careful when evaluating products you may see advertised or online. There is possible danger in using some herbal remedies. Some could damage your liver further. There are very few large, controlled scientific studies concerning herbals and supplements. The Food and Drug Administration does not regulate the manufactured quality of herbal supplements; herbal labels usually do not indicate dosage recommendations for PSC.

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 tiredness.
  • Depression: feelings of despair or hopelessness.
  • Pain: in right side or middle of abdomen towards rib cage. This is often termed right upper-quadrant (RUQ) pain. Pain may be of any intensity and may last for an indefinite period. Pain may extend to the shoulder blade.
  • Jaundice: yellowing of eyes and skin caused by excess bilirubin that the liver cannot process. May be accompanied by dark urine.
  • Chills and fever: signs of bacterial infections in bile ducts requiring immediate medical attention including administration of antibiotics.

Related problems: PSCers report additional symptoms: dark urine, variceal bleeding (spontaneous bleeding in the veins around the esophagus), ascites (fluid in belly), bacterial cholangitis, and hepatic encephalopathy (sometimes called HE). In addition patients may have night sweats, mental fog, nausea, vitamin deficiency, stool changes, weight loss, clotting problems, memory problems, hypertension, pancreatitis, cirrhosis, cholangiocarcinoma, and nail clubbing.


Personal Stories

Vitamin deficiency and PSC
It's recommended that patients with cholestatic liver diseases like primary biliary cirrhosis and primary sclerosing cholangitis do get checked for fat-soluble vitamin deficiencies such as vitamin D and A, and then take supplements to correct any deficiencies. But you have to be very careful not to over-dose. A good guide is this article which can be found by clicking here.

There is information on credible web sites (Mayo, WrongDiagnosis, NIDDK, ALF, etc.) that discusses PSC symptoms. The problem is that many of the symptoms listed are accompanied by others (especially if you have Crohn's or ulcerative colitis), making it difficult to tell what's going on.

Just as an example, let's say I'm experiencing fatigue, stiffness/pain in my back and neck. It comes and goes, but seems to be getting worse. Did I just overdo some activity (like baling hay!), is it a PSC flare, or am I experiencing ankylosing spondylitis (one of the possible extra-intestinal complications of ulcerative colitis)? Or is it just age (please, no!)?

Also, it's not uncommon at all for PSCers to have multiple autoimmune diseases, further complicating things (e.g., I have UC, PSC, asthma, eczema, arthritis).

Arne M.

Ascites and portal hypertension
Ascites (for people with liver disease) happens as a by-product of portal hypertension, (increased pressure in the vein system that carries blood from stomach, spleen & intestines to the liver) and cirrhosis. If you aren't in stage 3 or 4 and don't have cirrhosis, you most probably don't have ascites.

Besides doing the wave test (actually rolling from one side to the other and watching the water move in waves) or pushing on one side of your stomach and checking if your belly moves in waves, you can check your albumin level. Normal is 3.5 - 5.0; if your albumin falls lower than 3.5 you can get ascites and edema. Albumin is a protein that keeps fluid/water in your blood, if the level gets too low, fluid leaks out of your blood and into your system: ascites. Without the lower than normal albumin, I don't think you can get ascites and a lower albumin doesn't happen until cirrhosis. At least that's how it was explained to us.

Barb H.

Tips for travelers with PSC

All of your prescriptions should be in the original, labeled bottles. Consider asking your hepatologist for a recommendation of a hepatologist in the locale where you'll be visiting. Take along contact information for your medical team as well as information on your medical history, perhaps a summary from a physician or related papers.

A medic alert bracelet or "dog tag" with pertinent data is recommended. Take along a copy or summary of your records. Depending on your condition, you may choose to travel only where advanced health care is readily available; or at least be able to arrange for fast transport to an area that does have reputable medical facilities (medical evacuation insurance). Visit http://www.medjetassist.com for more information.

You may also want to consider travelers insurance for expensive trips. Just do a Google search; there are many companies offering inexpensive travel insurance. Medical evacuation insurance is recommended for some parts of the world.

Note: Southwest Airlines offers a generous ticket refund policy: "Unused, fully refundable tickets that do not carry restrictions may be applied toward future travel or refunded within one year from the date of issue." Thanks to PSCer Melanie S. for that information.

The Crohn's and Colitis Foundation's website has an excellent section on traveling with IBD, which would be useful to PSC patients. Click here to visit that site.

There are tips from the TSA on traveling and airport security. Click here for the website.

Arne M.