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.

Short Bowel Syndrome with Intestinal Failure (SBS-IF)

Short bowel syndrome with intestinal failure is a condition where individuals cannot absorb nutrients properly because they lack part of their intestine due to portions being surgically removed, parts missing since birth, or portions damaged and unable to function properly.

Symptoms

Symptoms may not occur for many patients with PSC. However, some patients may experience a variety of symptoms including:

Fatigue

Itching

Jaundice (yellowing of the eyes or skin)

Pain in the upper abdomen

Sleep disruptions and night sweats

Fever and chills

Diagnosis Journey

Learn more about how people can be diagnosed with primary sclerosing cholangitis.

When to see your doctor

Some patients may experience low fevers, chronic fatigue, or persistent itching sensations. Others may not present symptoms at all and learn about their diagnosis through a routine check-up or liver test. You should always speak with your doctor if you notice any changes in your symptoms or you develop new symptoms.

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.

If you are experiencing symptoms or challenges with your care, you may wish to seek a second opinion.

Specialists

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

Registered doctors

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

John Smith, MD

Gastroenterologist • Nashville, TN

Ongoing Research and Treatment Options

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

Current Clinical Trials

There are over 30 primary sclerosing cholangitis clinical trials actively recruiting participants. Search this site to find PSC clinical trials near you.

Visit Clinicaltrials.gov

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.

Principal Investigator Spotlight

Dr. Ziad H. Younes

Gastroenterologist and Director of Clinical Research at Gastro One

Sharing the Patient Experience in Clinical Trials Will Be the Key to Understanding and Developing Treatments for PSC

Read Dr. Younes's article where he discussed ongoing research for primary sclerosing cholangitis.

Advocacy Groups

Connect with fellow PSC patients and caregivers online and get further support through advocacy group resources.

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.

PSC Support App

The PSC Support app equips you with knowledge to get the care that’s right for you, when you need it. It has been designed by people living with PSC to help you live better with PSC.

PSC Support Private Facebook Group

Join the private PSC Support UK Facebook group to connect with others and learn about PSC.

PSC Support - Events

PSC Support holds online and in-person events throughout the year.

PSC Partners Seeking a Cure

A patient advocacy organization for patients with primary sclerosing cholangitis where they can get support from other PSC patients and share their experiences.

PSC Partners Seeking a Cure (Open) Support Group

A Facebook support group for PSC patients to increase the awareness of PSC. This Facebook group is an integral part of PSC Partners Seeking a Cure, a patient advocacy organization.

PSC Partners Seeking a Cure (Closed) Support Group

A private Facebook support group for PSC patients to increase the awareness of PSC. This Facebook group is an integral part of PSC Partners Seeking a Cure, a patient advocacy organization.

PSC Partners Seeking a Cure Blog

A blog for PSC patients which aims to provide varied viewpoints and offer advice, information, and support.

New Study Explores Unique Approach to Treat a Rare Liver Disease

Read this article about a new study that explores a different approach to treating primary sclerosing cholangitis.

Primary Sclerosing Cholangitis: Therapy with Synthetic Bile Acid Acts Directly on the Immune System

Visit this article to learn more about studies that demonstrated positive effects from administering synthetically produced bile acids and bile acid receptor agonists.

Resources

Find more resources below for you or a loved one.

Looking to learn more? Below are trusted medical sites where you can search for scholarly articles and information about PSC. Always speak with your doctor or medical professional about your findings.

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.

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.

American Liver Foundation

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

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. Learn more about PSC here.

Psychology Today
Find a mental health professional near you.

Primary Sclerosing Cholangitis and Social Security Disability
Read about how people living with PSC can file for social security disability.

Mental Health America (MHA)
Mental Health America is committed to promoting mental health and preventing mental illness through advocacy, research, education, and services.

Each patient has their own journey with PSC. Experiences shared in the podcasts and websites below should not be substituted for medical advice. Always speak to your doctor or a medical professional about your condition.

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.

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.

FAQs

What causes PSC?

There is no known cause, but PSC may potentially have genetic, autoimmune, and environmental origins associated with it.

How common is PSC?

Globally, one in every 10,000 people is diagnosed with primary sclerosing cholangitis.

How common is it for PSC patients to also have IBD?

Up to 90% of patients with PSC also have IBD.

Who is most at risk for PSC?

PSC affects men more than women. PSC can affect people of any age, however it is more frequently diagnosed in people between the ages of 30 and 40.

Can PSC be reversed?

Many PSC treatments have been studied, but none have been shown to reverse the liver damage caused by this condition.

When does a liver transplant become necessary for patients with PSC?

PSC patients will continue to experience a gradual loss of their liver function over time. If liver failure in patients with PSC becomes severe, a liver transplant may be necessary.

PatientWing gratefully acknowledges Dr. Ziad H. Younes for assistance in the review of this condition information. Updated April 2023.
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