Doctors primarily use Liver Function Tests (LFTs) to track enzyme levels and overall organ function. If the values are outside or above the normal range, this may indicate a liver problem, among other possible explanations, as discussed below:

• Alkaline Phosphatase (ALP): The most common marker for PSC; elevated levels usually indicate bile duct damage or obstruction.
• Gamma-Glutamyl Transferase (GGT): Often used alongside ALP to confirm that elevations are liver-related rather than bone-related.
• ALT and AST: These enzymes measure active inflammation and damage to liver cells (hepatocytes).
• Bilirubin: High levels indicate the liver is not properly clearing waste, which can lead to jaundice as the disease progresses.
• Albumin: A protein made by the liver; low levels can signal declining liver function or active inflammatory bowel disease (IBD).

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, and is not caused by alcohol consumption
• 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

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

• Pruritus or intense itching: Particularly on soles of feet or palms of hands, though it can occur anywhere, including in the eyes, ears, 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, vary in intensity, and may come and go.
• 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.

Although the average time from diagnosis to liver transplantation is 15-20 years, some patients need transplants sooner, and some never need a transplant.

• Abnormal Blood Tests:  Several blood tests, including bilirubin, creatinine, and International Normalized Ratio (INR), may become abnormal and may indicate whether a liver transplant is needed
• Ascites: Buildup of fluids in the abdomen
• Encephalopathy: Personality changes, confusion, and sleep disturbances caused by a buildup of toxins, such as ammonia, in the blood
• Varices: Swollen veins prone to bleeding, usually in the esophagus. When bleeding occurs, it can cause vomiting of blood or passage of black, tarry stool. Medical attention should be sought immediately when this happens.
• Splenomegaly: An enlarged spleen
• Jaundice: Yellowing of eyes and skin caused by excess bilirubin

• Bacterial Cholangitis (a severe, often acute, infection of the bile ducts)
• Biliary Stone Disease (hardened deposits of bile components, mostly cholesterol, forming in the gallbladder or ducts)
• 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)
• Night sweats, nausea
• Vitamin deficiency of fat-soluble vitamins A, D, E, or K
• Stool changes
• Weight loss
• Osteopenia (mild loss of bone density) or osteoporosis (advanced, severe bone loss with risk of bone fractures)

At this time, there are no FDA-approved medications for the treatment and cure of PSC, 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 in our FAQ section.)
• At this time, the only definitive treatment is a liver transplant. Some PSC patients live long and productive lives with the disease and never need a transplant. Others end up needing a transplant, and they return to health. In some cases, the PSC returns post-transplant. This is called recurrent PSC (rPSC). Some PSC patients have undergone multiple liver transplants.

Clinical Trials

There is an increasing number of PSC clinical trials underway. PSC Partners encourages everyone to research and discuss all potential treatments and medications, including participation in clinical trials, with their health care professionals.

Visit our Clinical Trials page to learn more and view of this of current trials taking place.