Primary Sclerosing Cholangitis: What You Need to Know About This Rare Liver Disease
Published: Mar 07, 2024
Primary sclerosing cholangitis (PSC) is a chronic liver disease that causes inflammation and scarring of the bile ducts. While rare, PSC can lead to serious complications and may require a liver transplant.
Contents
What Causes PSC?
The exact cause of PSC is unknown, but it's believed to be an autoimmune condition where the body's immune system mistakenly attacks the bile ducts. PSC is closely linked to inflammatory bowel diseases like ulcerative colitis. Genetic factors may also play a role in developing PSC.
Signs and Symptoms
Early on, PSC may not cause any noticeable symptoms. As the disease progresses, common symptoms include fatigue, itchy skin, jaundice (yellowing of the skin/eyes), abdominal pain, and unexplained weight loss. Some patients experience episodes of fever, chills and abdominal pain due to recurrent infections of the bile ducts.

Diagnosis and Testing
PSC is usually diagnosed through a combination of blood tests, imaging studies, and sometimes a liver biopsy. Blood tests can show elevated liver enzymes and signs of cholestasis. Imaging tests like MRI or ERCP can visualize the characteristic 'beaded' appearance of the bile ducts. A liver biopsy may be done to confirm the diagnosis and assess the extent of liver damage.
Treatment Options
There is no cure for PSC, so treatment focuses on managing symptoms and slowing disease progression. Medications may be used to relieve itching and other symptoms. Endoscopic procedures can open up narrowed bile ducts. For advanced cases, liver transplantation may be necessary. Ongoing monitoring is important to watch for complications like bile duct cancer.
Frequently Asked Questions
About 70-80% of PSC patients also have IBD, most commonly ulcerative colitis.
PSC is progressive, but the rate varies widely between individuals.
There are no known ways to prevent PSC.
PSC isn't directly inherited, but genetic factors may increase risk.
PSC affects about 1 in 10,000 people.
Key Takeaways
While PSC is a serious condition, proper management and monitoring can help many patients maintain their quality of life for years.
References
- Chapman R, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51(2):660-678.
- Lindor KD, et al. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol. 2015;110(5):646-659.
- Eaton JE, et al. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management. Gastroenterology. 2013;145(3):521-536.
This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic. Always discuss health information with your healthcare provider.
AI Doctor Visit Required
Appointments available 24/7
15-min consultation. No hidden costs.
AI Doctor Visit Required
For safety reasons we have been forced to end this consultation.
If you believe this is a medical emergency please call 911 or your local emergency services immediately.
If you are experiencing emotional distress, please call the the Suicide & Crisis Lifeline at 988 or your local crisis services immediately.
Contact us
You can also email us at help@doctronic.ai
We aim to reply within 5-7 days
How likely are you to recommend Doctronic to friends or family?