Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis is the inflammation and scarring of the bile ducts (tubes carrying bile) located inside and outside of the liver. Bile is digestive fluid that is transported from your liver to your small intestine through the bile ducts. PSC can cause the bile ducts to narrow and lead to build up of bile in the liver. This can cause progressive liver damage and over time in severe cases can lead to liver failure and need for liver transplant. It can also increase the risk of infection in bile duct, liver cancer, and bile duct cancer.

Fatigue

Itching

Pain in the upper abdomen

Sleep disruptions and night sweats

Jaundice (yellowing of skin and eyes)

Fever and chills

Common tests used in diagnosis and monitoring of PSC:

Magnetic Resonance Cholangiopancreatography (MRCP): A MRCP is a special type of MRI that can be used to create detailed images of the liver and bile ducts.

Liver Function Blood Test: This test checks the efficiency of your liver and liver enzyme levels.

Endoscopic Retrograde Cholangiopancreatography (ERCP): This is an X-ray of the bile ducts and may be used in addition to an MRI. During an ERCP, a flexible tube is inserted down the throat and is used to inject dye into the area of connection between the bile duct and small intestine. An ERCP is used after an MRCP shows a blocked duct. An ERCP can also be performed to dilate and stent dominant strictures.

Liver Biopsy: A portion of the liver is removed and tested in a laboratory. This test is only used in special cases when the diagnosis is still uncertain.

Additional Imaging: Imaging such as an ultrasound and CT scan can be done to screen for cancer. Fibroscans can also be done to follow and monitor degree of liver scarring/liver damage.

Explore the treatment options available for PSC.

Treatments for Itching

There are no approved treatments for itching but some patients may find relief through the following options. Always speak with your doctor to determine what might be right for you.

Antibiotics: The most common antibiotic to help relieve itching in PSC is called rifampin, which is a drug used to treat tuberculosis. Rifampin has been shown to relieve itching by activating receptors in a patient's gut and liver.

Bile Acid Sequestrants: Cholestyramine is a common bile acid sequestrant that reduces high cholesterol and can improve itching in PSC.

Ursodeoxycholic Acid: This medication can help relieve itching caused by liver disease by fighting bile acids that build up.

Treatments for Bile Duct Blockages

Stents: Stents are placed in the bile ducts that are blocked to keep them open.

Balloon Dilation: A procedure to help open blockages in the bile ducts.

Liver Transplant

In the later stages of PSC, there might be a need for a living or cadaveric liver transplant.

Liver Transplant: A liver transplant is for patients who are in liver failure or have severe PSC complications. The patient’s diseased liver will be replaced with a healthy liver from a donor.

Ongoing Research

PSC is a rare disease and currently, there are no approved treatments by any regulatory agency.

Current Research Studies

There are over 30 primary sclerosing cholangitis clinical trials actively recruiting participants. Visit ClinicalTrials.gov to find PSC clinical trials near you.

Featured Study

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Resources

Find an online community of fellow patients, caregivers, and advocates below
as well as some other general resources!

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PSC Support

PSC Support is the only UK patient organisation dedicated to improving the lives of people affected by PSC. PSC Support funds PSC research and provides information and support to people living with PSC.

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National Organization for Rare Disorders (NORD)

NORD's mission is to improve care, advance research, and drive policy for patients and families affected by rare diseases.

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American Liver Foundation

ALF’s mission is to promote education, advocacy, support services and research for the prevention, treatment and cure of liver disease.

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PubMed

PubMed is a free resource supporting the search and retrieval of biomedical and life sciences literature with the aim of improving health–both globally and personally.

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European Reference Network for Rare Liver Diseases (ERN RARE-LIVER)

ERN RARE-LIVER is a Europe-wide network for centres of excellence in the clinical management of rare liver disease in adults and in children. The network aims to improve the standards of clinical knowledge and of patient care in rare liver diseases throughout Europe.

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How to Deal When the Sh*t Gets Real Episode 39 - Invictus Life with Russell Creed

On this episode of How to Deal When the Sh*t Gets Real, Russell Creed shares his journey with primary sclerosing cholangitis, his liver transplant experience, and how he helps others find purpose and mindset.

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The Comical Colon

The Comical Colon website was created by Jenna Ziegler, a writer and chronic illness advocate living with ulcerative colitis, arthritis, and primary sclerosing cholangitis. On this website, Jenna writes about her own chronic illness journey and offers advice and support to others on their own chronic illness journeys.

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