What is primary sclerosing cholangitis?
Primary sclerosing cholangitis (PSC), sometimes referred to as sclerosing cholangitis or PSC, is a rare condition that affects the bile ducts. Bile ducts are thin tubes that move bile from the liver and gallbladder into the small intestine. Bile is the fluid made and released by the liver that helps with digestion and is stored in the gallbladder. PSC occurs when inflammation in the bile ducts leads to scarring and narrowing of the ducts. This results in bile building up in the liver, which causes serious liver damage over time that can lead to liver failure.
Primary sclerosing cholangitis is treated by doctors who specialize in liver disorders called gastroenterologists and hepatologists. Specialists at Penn Medicine are among the leading providers in the southeastern Pennsylvania and South Jersey region who are dedicated to diagnosing and treating conditions like primary sclerosing cholangitis. In addition, treating liver disorders often involves coordination among different specialists to ensure the best outcome. Our team consists of pathologists, oncologists, endoscopy specialists, and surgeons. We focus on learning about you to find the best ways to eliminate or successfully manage your condition.
Primary sclerosing cholangitis symptoms
PSC often has no symptoms until an advanced stage, but it’s sometimes discovered during routine exams or testing for other health issues.
Early symptoms
Early symptoms of primary sclerosing cholangitis can include:
- Fatigue
- Itching
- Pain in the upper part of your abdomen
- Yellowing of the skin and whites of the eyes, called jaundice
Advanced symptoms
Advanced symptoms of primary sclerosing cholangitis can include:
- Chills
- Enlarged liver
- Enlarged spleen
- Fever
- Sweating
- Unexplained weight loss
What causes primary sclerosing cholangitis?
The cause of PSC is unknown, but research has shown the following information about the condition:
- About 70 percent of people with PSC also have an inflammatory bowel disease such as ulcerative colitis, but the link between the conditions is unclear.
- You are at an increased risk of getting PSC if you have a blood relative who has it.
- An immune system reaction to an infection or toxin may trigger PSC, meaning the immune system targets the body’s tissues and organs.
Diagnosing primary sclerosing cholangitis
To diagnose PSC, your Penn Medicine provider will ask about your symptoms, review your medical history, and do a physical exam. In addition to blood tests, your doctor may also recommend one or more of the following tests:
Primary sclerosing cholangitis treatments
While there isn’t a cure for primary sclerosing cholangitis, Penn Medicine specialists provide expert care for patients with gastrointestinal and digestive system disorders like PSC. Your provider may talk with you about lifestyle changes that help care for your liver, such as avoiding alcohol and illegal drugs, following the directions on medications, and getting vaccines. Additionally, your doctor may recommend the following treatments.
Primary sclerosing cholangitis risk factors
The following factors may increase your risk of PSC:
- Age: It can occur at any age but is most often diagnosed in people 30 to 40 years old.
- Inflammatory bowel disease: It’s more likely to occur in people who have inflammatory bowel disease.
- Heritage: It occurs more often in people with Northern European heritage.
- Sex: It occurs more often in people assigned male at birth.
Your path to wellness with Penn Medicine
At Penn Medicine, our mission is to relieve your symptoms and improve your overall well-being. We’re dedicated to guiding you through every phase of your medical journey to make sure you get the highest level of care every step of the way. Everyone's experience with primary sclerosing cholangitis is different, so it's essential to work closely with your care team to find the right path for you.