Primary Sclerosing Cholangitis (PSC)
What is Primary Sclerosing Cholangitis (PSC)?
Primary sclerosing cholangitis is a chronic liver disease that causes inflammation and scarring in the bile ducts. Bile ducts are small tubes that carry bile, a digestive fluid made by your liver, to your small intestine. When these ducts become inflamed and scarred, bile cannot flow properly.
Over time, the scarring can block the bile ducts completely. This causes bile to build up in the liver, which damages liver cells and leads to cirrhosis. Cirrhosis is severe scarring of the liver that stops it from working properly. PSC progresses slowly, often over many years.
Most people with PSC also have inflammatory bowel disease, especially ulcerative colitis. The condition affects men more often than women and typically appears between ages 30 and 40. Without treatment, PSC can lead to liver failure and may require a liver transplant.
Symptoms
- Intense itching of the skin
- Fatigue and weakness
- Yellowing of the skin and eyes, called jaundice
- Pain in the upper right side of the abdomen
- Fever and chills from bile duct infections
- Unintended weight loss
- Night sweats
- Dark urine
- Pale or clay-colored stools
Many people with early PSC have no symptoms at all. The condition is often discovered through abnormal liver blood tests done for other reasons. Symptoms typically appear as the disease progresses and bile ducts become more blocked.
Concerned about Primary Sclerosing Cholangitis (PSC)? Check your levels.
Screen for 1,200+ health conditions
Causes and risk factors
The exact cause of primary sclerosing cholangitis is unknown. Researchers believe it is an autoimmune condition, meaning the immune system mistakenly attacks healthy bile duct cells. Genetic factors likely play a role, as PSC tends to run in families. Environmental triggers may also contribute, though none have been clearly identified.
About 70% of people with PSC also have inflammatory bowel disease, usually ulcerative colitis. This strong link suggests shared immune system problems. Other risk factors include being male, having a family history of PSC, and being of Northern European descent. Most people develop PSC between ages 30 and 40, though it can occur at any age.
How it's diagnosed
PSC is diagnosed through a combination of blood tests and imaging studies. Blood tests can reveal elevated liver enzymes, especially alkaline phosphatase and gamma-glutamyl transferase, which indicate bile duct problems. Bilirubin levels also rise as the disease progresses. Rite Aid's testing panel includes these key markers to help monitor liver and bile duct health.
If blood tests suggest PSC, your doctor will order imaging tests to look at your bile ducts. The most common test is MRCP, a special type of MRI that shows the bile ducts clearly. Sometimes a procedure called ERCP is needed to examine the ducts more closely and take tissue samples. A liver biopsy may also be performed to assess the extent of liver damage.
Treatment options
- Ursodeoxycholic acid medication to improve bile flow and protect liver cells
- Antibiotics to treat bile duct infections when they occur
- Medications to control itching, such as cholestyramine
- Vitamin supplements, especially fat-soluble vitamins A, D, E, and K
- Regular monitoring through blood tests and imaging every 6 to 12 months
- Screening for bile duct cancer, which occurs in 10% to 15% of PSC patients
- Treatment of inflammatory bowel disease if present
- Liver transplant for advanced disease with liver failure
- Avoiding alcohol completely to reduce liver stress
- Maintaining a healthy weight and eating a balanced diet
Concerned about Primary Sclerosing Cholangitis (PSC)? Get tested at Rite Aid.
- Simple blood draw at your nearest lab
- Results in days, not weeks
- Share results with your doctor
Frequently asked questions
Life expectancy varies widely depending on disease severity and progression rate. Without a liver transplant, median survival from diagnosis is 12 to 18 years. Some people live much longer with mild disease, while others progress more quickly. Regular monitoring and treatment can help extend life expectancy and maintain quality of life.
There is no cure for PSC except liver transplant. Medications can help manage symptoms and slow disease progression. Ursodeoxycholic acid may improve bile flow and protect the liver. A liver transplant can cure the liver disease, but PSC sometimes returns in the transplanted liver.
Most doctors recommend blood tests every 3 to 6 months to monitor disease progression. These tests check liver enzymes like alkaline phosphatase and GGT, along with bilirubin levels. More frequent testing may be needed if your disease is progressing or if you develop new symptoms. Regular monitoring helps catch complications early.
PSC can run in families, suggesting a genetic component. However, most people with PSC have no family history of the condition. Having a first-degree relative with PSC increases your risk about 100 times compared to the general population. Genetic factors likely interact with environmental triggers to cause the disease.
About 70% of people with PSC also have inflammatory bowel disease, usually ulcerative colitis. The connection suggests shared immune system abnormalities. Having ulcerative colitis does not mean you will develop PSC, as only 2% to 7% of ulcerative colitis patients get PSC. Both conditions require regular monitoring and coordinated treatment.
No specific diet can cure or stop PSC progression. However, eating well helps maintain liver health and overall strength. Focus on nutrient-dense whole foods and avoid alcohol completely. Some people with PSC have trouble absorbing fats and fat-soluble vitamins, so supplements may be needed. Work with a dietitian familiar with liver disease for personalized guidance.
Alkaline phosphatase is an enzyme that rises when bile ducts are blocked or damaged. In PSC, inflammation and scarring prevent normal bile flow, causing this enzyme to leak into the bloodstream. Levels often range from 2 to 10 times normal. Tracking alkaline phosphatase over time helps doctors monitor disease activity and progression.
Yes, PSC significantly increases the risk of bile duct cancer, also called cholangiocarcinoma. This cancer develops in 10% to 15% of people with PSC. The risk of colon cancer is also higher, especially in those with ulcerative colitis. Regular screening with imaging and colonoscopy is essential for early detection.
Itching occurs because bile salts build up in the bloodstream and skin when bile ducts are blocked. This symptom, called pruritus, can be severe and affect sleep and quality of life. Medications like cholestyramine can help by binding bile salts in the intestines. Some people find relief with antihistamines or other prescription medications.
Liver transplant is considered when PSC causes liver failure or severely impacts quality of life. Signs include persistent jaundice, fluid buildup in the abdomen, confusion from liver toxins, or recurrent bile duct infections. Transplant can also be needed if bile duct cancer is detected early. Survival rates after transplant are good, with 5-year survival above 80%.