Primary Sclerosing Cholangitis (PSC)
Progressive scarring of the bile ducts
Quick Facts
- Type: Chronic liver and bile duct disease
- Effect: Narrowing and scarring of bile ducts
- Strong link: Inflammatory bowel disease
- Possible outcome: Liver scarring (cirrhosis)
Overview
Primary sclerosing cholangitis (PSC) is a long-term disease of the bile ducts, the tubes that carry bile from the liver to the intestine to help digest fats. In PSC, ongoing inflammation causes the ducts to become scarred and narrowed. As the narrowing worsens, bile backs up in the liver, which can gradually injure liver tissue.
Over many years, this damage can lead to liver scarring (cirrhosis) and reduced liver function. PSC has a notably strong association with inflammatory bowel disease, especially ulcerative colitis, and many people with PSC also have bowel disease. The condition progresses at different rates in different people, and care focuses on managing symptoms, treating complications, and monitoring for problems over time.
Symptoms
In its early stages PSC often causes no symptoms and may be found through abnormal liver blood tests. As it progresses, symptoms can include:
- Fatigue
- Itching of the skin
- Yellowing of the skin and eyes (jaundice)
- Pain in the upper right abdomen
- Fever and chills, especially if the ducts become infected
- Unintended weight loss
Episodes of fever with jaundice and abdominal pain can signal an infection of the bile ducts (cholangitis), which needs prompt medical attention.
Causes
The exact cause of PSC is not known. It is widely thought to be an immune-related disease, in which the body's immune system contributes to inflammation and scarring of the bile ducts, in people with a genetic predisposition. Environmental factors may also play a role.
One of the most striking features of PSC is its close link with inflammatory bowel disease, particularly ulcerative colitis. The two conditions often occur together, although either can appear first, and the bowel and liver diseases can follow independent courses. PSC is not caused by alcohol or by infection of the ducts itself.
Risk Factors
- Having inflammatory bowel disease, especially ulcerative colitis
- Being male, as PSC is somewhat more common in men
- Being diagnosed in young to middle adulthood
- A family history of the disease in some cases
Diagnosis
PSC is often suspected from abnormal liver tests and confirmed with imaging of the bile ducts:
- Blood tests: Liver function tests, often showing a pattern suggesting blocked bile flow.
- MRCP: A special MRI scan of the bile ducts that shows the characteristic narrowing and beaded appearance; this is the main diagnostic test.
- ERCP: An endoscopic procedure that can image the ducts and treat narrowings when needed.
- Liver biopsy: Occasionally used to assess the degree of liver damage.
Because PSC and bowel disease are linked, a colonoscopy is often recommended to check for inflammatory bowel disease.
Treatment
There is no medication proven to cure PSC, so treatment focuses on managing symptoms and complications:
- Itch relief: Medications to reduce troublesome itching.
- Treating infections: Antibiotics for episodes of bile duct infection (cholangitis).
- Opening blocked ducts: ERCP can stretch or stent significant narrowings to improve bile flow.
- Nutritional support: Including fat-soluble vitamins, which can become deficient.
- Monitoring: Regular surveillance because PSC raises the risk of certain cancers of the bile ducts, gallbladder, and, with bowel disease, the colon.
- Liver transplant: The main option for advanced liver failure and can be very effective.
Because PSC progresses at very different rates in different people, ongoing monitoring is a central part of care. Regular blood tests, imaging, and recommended cancer surveillance help detect complications early, when they are most treatable. People with PSC often work with a team that may include a liver specialist and, when bowel disease is present, a gastroenterologist, so that both the liver and the bowel are managed together over the long term.
Prevention
- PSC cannot currently be prevented
- Regular follow-up and recommended cancer screening help detect problems early
- Prompt treatment of bile duct infections protects the liver
- Avoiding alcohol and other liver stressors supports overall liver health
- Keeping vaccinations such as hepatitis A and B up to date is often advised
When to See a Doctor
See a doctor if you have persistent fatigue, itching, or yellowing of the skin or eyes, or if routine tests show abnormal liver results. Seek urgent care for:
- Fever and chills with yellowing skin and upper-right abdominal pain, which may signal a bile duct infection
- Vomiting blood or black, tarry stools
- Confusion or marked drowsiness
- Significant swelling of the abdomen or legs
Frequently Asked Questions
What is the connection between PSC and ulcerative colitis?
PSC is strongly linked with inflammatory bowel disease, especially ulcerative colitis, and many people with PSC also have bowel disease. Either condition can appear first, and they can progress independently, so doctors usually check for bowel disease in people with PSC.
Can primary sclerosing cholangitis be cured?
There is currently no medication proven to cure PSC. Treatment focuses on relieving symptoms, treating bile duct infections and blockages, and monitoring for complications. For advanced liver failure, a liver transplant can be an effective treatment.
What are the warning signs of a bile duct infection in PSC?
Fever and chills together with yellowing of the skin and pain in the upper right abdomen can signal a bile duct infection called cholangitis. This needs prompt medical attention and often treatment with antibiotics.
Does PSC increase cancer risk?
Yes. PSC raises the risk of cancers of the bile ducts and gallbladder, and, in people who also have inflammatory bowel disease, the risk of colon cancer. Regular surveillance is recommended to catch problems early.
How is PSC diagnosed?
It is usually suspected from abnormal liver blood tests and confirmed with an MRI scan of the bile ducts called MRCP, which shows the characteristic narrowing. A colonoscopy is often done because of the link with bowel disease.
References
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Primary sclerosing cholangitis.
- Mayo Clinic. Primary sclerosing cholangitis.
- MedlinePlus, U.S. National Library of Medicine. Sclerosing cholangitis.
- American Liver Foundation. Primary sclerosing cholangitis.