Primary Sclerosing Cholangitis (PSC) is a chronic liver disease in which bile ducts of the liver become damaged and blocked, leading to bile buildup, which can severely impact liver health over time.
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Primary Sclerosing Cholangitis (PSC) is a chronic liver disease characterized by progressive inflammation and scarring of the bile ducts of the liver. These ducts are responsible for carrying bile, a liver-produced fluid essential for digesting fats, from the liver to the small intestine. In PSC, bile ducts become damaged and blocked, leading to bile buildup, which can severely impact liver health over time.
PSC requires multidisciplinary care with coordination. At UC Health, we have experts in liver disease, IBD, and advanced endoscopy to provide world-class care for PSC patients throughout their entire disease course.
Inflammation and Scarring (Fibrosis): PSC causes persistent inflammation and scarring in the bile ducts, leading to bile duct blockages and obstructed bile flow.
Bile Duct Obstruction: As the ducts become blocked, bile accumulates in the liver, which can result in biliary obstruction and further liver damage.
Liver Function Impact: Obstructed bile flow and bile buildup harm liver tissue, increasing the risk of liver cirrhosis, liver failure, and even cholangiocarcinoma (bile duct cancer).
As PSC progresses, the liver undergoes increasing damage, leading to potential complications:
Cirrhosis and Liver Failure: Ongoing bile duct obstruction can lead to liver cirrhosis, where healthy liver tissue is replaced by scar tissue, potentially resulting in liver failure.
Bacterial Cholangitis: Blocked bile ducts can make PSC patients prone to infections, such as bacterial cholangitis.
Cancer Risks: PSC is associated with an elevated risk of cholangiocarcinoma (bile duct cancer) and colon cancer in patients with inflammatory bowel disease (IBD), especially ulcerative colitis.
PSC may progress gradually, with symptoms often varying in severity and appearing over time. Key symptoms include:
Itching (Pruritus): Severe itching, often caused by bile buildup.
Jaundice: Yellowing of the skin and eyes due to liver dysfunction.
Fatigue and Abdominal Pain: Persistent tiredness and discomfort, especially in the upper right abdomen, related to liver and bile duct inflammation.
Weight Loss and Enlarged Liver: Unintentional weight loss and potential hepatomegaly (enlarged liver) as liver damage advances.
Recognizing these symptoms early and consulting with a healthcare provider can lead to prompt evaluation and management.
Diagnosing PSC requires a thorough approach, including a review of symptoms, medical history, and specialized testing to confirm the presence and extent of bile duct damage.
Blood Tests: Liver function tests check for elevated liver enzymes, and autoimmune markers may help identify PSC as part of autoimmune disease.
Magnetic Resonance Cholangiopancreatography (MRCP): This imaging technique provides detailed images of the bile ducts, helping to detect bile duct narrowing, blockages, and liver damage.
Endoscopic Retrograde Cholangiopancreatography (ERCP): Used for diagnostic and therapeutic purposes, ERCP can help identify and treat bile duct strictures.
Liver Biopsy: Sometimes performed to assess liver tissue for fibrosis and rule out other liver diseases, such as primary biliary cholangitis or autoimmune hepatitis.
Regular follow-ups and imaging allow healthcare providers to track disease progression and manage complications.
The exact cause of PSC remains unknown, but several factors appear to increase risk:
Genetic Predisposition: A family history of PSC or other autoimmune diseases may increase the likelihood of developing the condition.
Inflammatory Bowel Disease: There is a strong association between PSC and IBD, particularly ulcerative colitis.
Potential Environmental Triggers: Certain infections or environmental exposures may also play a role in disease development.
While PSC has no cure, treatment focuses on managing symptoms, preventing complications, and preserving liver function.
Ursodeoxycholic Acid (UDCA): Often used to improve bile flow and reduce bile acid buildup, although it does not halt disease progression.
Immunosuppressants: Drugs like azathioprine may help manage autoimmune responses in patients with concurrent autoimmune hepatitis or IBD.
Endoscopic Retrograde Cholangiopancreatography (ERCP): Used to dilate strictures or place stents in blocked bile ducts, ERCP can relieve symptoms related to bile duct blockages.
Percutaneous Transhepatic Cholangiography (PTC): A radiological procedure to access the bile ducts and treat severe obstructions.
Liver Transplantation: For patients with advanced liver disease or liver failure, a transplant can be a life-saving option.
Regular monitoring and symptom management are essential for PSC patients. Healthcare providers may recommend lifestyle changes, such as limiting alcohol, adopting a balanced diet, and avoiding substances that strain the liver.
Living with PSC involves ongoing management and support, with lifestyle adjustments and regular check-ups essential for quality of life.
Healthy Diet: Consuming a liver-friendly diet rich in fruits, vegetables, and lean protein can support liver health.
Exercise and Stress Management: Physical activity and mindfulness techniques can help manage symptoms like fatigue and stress.
Patient Advocacy Groups: Organizations like PSC Partners Seeking a Cure provide education and community support.
Clinical Trials: Participation in clinical trials offers access to new treatment options and contributes to PSC research.
By staying informed and engaging with a healthcare team, PSC patients can actively manage their condition and improve their quality of life.
Here at UC Health, you will have access to the latest medical therapies and clinical trials. Ongoing research and clinical trials continue to explore the underlying causes and potential treatments for PSC, with studies focusing on:
Genetic and Autoimmune Mechanisms: Research is examining genetic links and autoimmune processes to develop targeted therapies.
Emerging Therapies: New treatments under investigation, including targeted medications, aim to slow disease progression and manage symptoms more effectively.
With these efforts, the future of PSC management holds promise for improved outcomes and more personalized care options.
At UC Health, we lead the region in scientific discoveries and embrace a spirit of purpose – offering our patients and their families something beyond everyday healthcare. At UC Health, we offer hope.