Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic disease that slowly causes long-term damage to the bile ducts.

your quick guide to this page

what is primary sclerosing cholangitis?: Learn what defines primary sclerosing cholangitis and what its general consequences are.
fast facts: Get a quick overview of primary sclerosing cholangitis through statistics and bite-sized facts.
symptoms: Learn about the symptoms of primary sclerosing cholangitis, how to recognize them, and what to do if you experience them.
lifestyle impacts: Find out how primary sclerosing cholangitis impacts your lifestyle.
what can I do?: Discover management and treatment strategies for primary sclerosing cholangitis.
additional resources: Find useful links, education, articles, recipes and more that can help with primary sclerosing cholangitis.

what is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a chronic disease that affects the bile ducts which are small channels that carry bile between organs in the biliary system, including the liver, gallbladder, and small intestine. In PSC, ongoing inflammation (cholangitis) causes the bile ducts to become irritated and damaged over time. This can lead to scarring, known as strictures, which narrow the ducts and restrict the normal flow of bile. When bile cannot flow properly, it can build up and gradually damage the liver, and bile toxins may leak into the bloodstream.

As PSC progresses, scar tissue (fibrosis) increases in the liver. In early stages, scarring is minimal and limited to small areas, but it can extend and begin to connect over time. In advanced stages, scarring can lead to cirrhosis, which is permanent liver damage.

fast facts

Primary sclerosing cholangitis affects 1 to 16 in every 100, 000 people.
Men are more likely to have primary sclerosing cholangitis than women.
The average age of diagnosis for patients with primary sclerosing cholangitis is 40 years old.
80% of people with primary sclerosing cholangitis also have inflammatory bowel disease (IBD). PSC also can occur in people with Crohn’s disease, though less commonly.
People with primary sclerosing cholangitis have a 10-20% chance of developing bile duct cancer.

symptoms

Primary sclerosing cholangitis is unpredictable and not well understood. A person can have the disease for many years before symptoms develop. When a patient does experience symptoms they may include:

The symptoms of PSC change as the disease progresses.
- Fatigue
- Pain in the upper right side of your abdomen
- Itchy skin (pruritus)
- Fever or chills
- Jaundice
- Dark urine or pale stools
Advanced symptoms:
- Swollen abdomen
- Enlarged liver
- Enlarged spleen
- Vomiting blood or having black stool

If a person who suffers from primary sclerosing cholangitis develops fever and chills, they should go to the hospital immediately. Eventually, in the advanced stages of PSC, cirrhosis may develop with signs of liver failure.

lifestyle impacts

People living with primary sclerosing cholangitis (PSC) often experience chronic fatigue, which can affect daily life. While PSC cannot be prevented, healthy lifestyle choices can help manage symptoms, support overall well-being, and reduce additional strain on the liver.

To support your liver health, consider:

Avoiding alcohol
Quitting smoking, with support from your healthcare provider
Eating a balanced diet with lean proteins, whole grains, fruits, and vegetables
Managing stress
Getting enough sleep
Staying physically active with regular, moderate to vigorous exercise

Related Conditions

PSC is often associated with other autoimmune diseases. Many people with PSC may also develop:

Inflammatory bowel disease (IBD)
Celiac disease
Thyroid disease
Type 1 diabetes
Autoimmune hepatitis
Autoimmune pancreatitis

Potential Complications

PSC can lead to a range of complications, particularly as the disease progresses:

Digestive and metabolic complications: Blocked bile ducts can interfere with digestion and prevent proper absorption of fats and fat-soluble vitamins (A, D, E, and K). This may lead to:
- Diarrhea and fatty stools
- Malnutrition
- Easy bruising and bleeding (vitamin K deficiency)
- Bone conditions such as osteomalacia or osteoporosis (vitamin D deficiency)
- Vision issues, especially in low light
Portal hypertension: Scarring of the liver (cirrhosis) can increase pressure in the portal vein, leading to swollen veins in the digestive tract that may rupture and cause internal bleeding.
Infections: Blocked bile ducts increase the risk of serious infections, which may cause fever, abdominal pain, or sepsis and often require urgent medical treatment.
Increased cancer risk: Advanced PSC is linked to a higher risk of certain cancers, including bile duct, liver, gallbladder, and colorectal cancer (particularly in those with IBD).
Liver damage and failure: Ongoing inflammation can lead to cirrhosis and progressive loss of liver function over time.
Bone and vitamin deficiencies: PSC may lead to weakened bones (osteoporosis) and deficiencies in vitamins A, D, E, and K due to impaired bile flow.

what can I do?

prevention

There is currently no known way to prevent primary sclerosing cholangitis (PSC). The exact cause is not fully understood, but it is believed to involve a combination of factors, including:

Genetics
Immune system problems
Changes in the gut microbiome, the community of microorganisms living in the intestines, may play a role in liver health. These microbes normally support digestion and help protect the body, but when this balance is disrupted, it can contribute to liver disease.
Environmental factors

Research on PSC continues to better understand these contributing factors.

Risk factors for PSC

Sex: PSC occurs more often in men than women.
Age: PSC can occur at any age, but it’s most often diagnosed in middle-aged adults. Being between 30 and 60
Having inflammatory bowel disease most often ulcerative colitis: About 5% to 7% of people with some form of IBD, such as ulcerative colitis or Crohn’s disease, also have PSC.
Geographical location: The number of diagnoses of PSC is higher in Northern Europe and North America than in other parts of the world.
Genes: Certain gene variations may play a role in the risk and development of the disease.

diagnosis

Since many patients with PSC do not experience any symptoms, diagnosis is often found through routine blood tests or by accident when testing for other conditions. Common tests for PSC include:

Blood test: high levels of alkaline phosphatase or indicate an immune response is happening. Having high white blood cells is usually a sign of infection in your liver.
Liver function test: look for high levels of certain liver enzymes. High levels of alkaline phosphatase may indicate PSC.
Imaging test:
- Magnetic resonance cholangiopancreatography (MRCP): This test uses magnetic resonance imaging (MRI) to produce detailed pictures of the biliary tree (your liver, gallbladder and bile ducts). This is the first-line imaging test for PSC because it’s non-invasive and avoids radiation exposure.

treatment

There currently is no known specific treatment for primary sclerosing cholangitis. However, symptoms of this disease can be treated with varying success by antibiotics, vitamins, and medications to control itching and improve bile flow. When the disease has progressed to liver failure, liver transplantation may be considered.

additional resources

Here are a few questions to ask your doctor or medical team:

What is the status of my liver?
What treatments do you recommend to slow the progression of the disease?
How can I relieve the symptoms I am experiencing?
Will I need a liver transplant?