Overview
Sclerosing cholangitis is a serious liver disease that affects the bile ducts — the tubes that carry bile from the liver to the small intestine. In this condition, the bile ducts become inflamed and scarred over time, causing them to narrow and eventually block the flow of bile. When bile cannot drain properly, it builds up in the liver and causes damage. There are two main forms: primary sclerosing cholangitis (PSC), which is the most common type and is often linked to inflammatory bowel disease, and secondary sclerosing cholangitis, which is caused by a known trigger such as infection, surgery, or injury to the bile ducts. Over time, the scarring and blockage can lead to serious complications including liver cirrhosis (severe scarring of the liver), liver failure, and an increased risk of bile duct cancer (cholangiocarcinoma). Common symptoms include fatigue, itching, yellowing of the skin and eyes (jaundice), abdominal pain, and recurring infections of the bile ducts called cholangitis episodes. There is currently no medication proven to stop or reverse the disease. Treatment focuses on managing symptoms, preventing complications, and treating infections. In advanced cases, a liver transplant may be the only life-saving option. Research into new therapies is ongoing, and patients are encouraged to be followed by a specialist experienced in liver disease.
Key symptoms:
Persistent fatigue and low energyItching of the skin (pruritus), often severeYellowing of the skin and whites of the eyes (jaundice)Pain or discomfort in the upper right abdomenFever and chills, especially during bile duct infectionsDark urinePale or greasy stoolsUnintended weight lossSwelling of the abdomen due to fluid buildup (ascites)Easy bruising or bleedingBone pain or increased risk of fractures due to poor vitamin absorptionRecurring episodes of bacterial infection in the bile ducts (cholangitis)
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
5 eventsFondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico — NA
Zydus Therapeutics Inc. — PHASE1
IRCCS Azienda Ospedaliero-Universitaria di Bologna
Medical University of Graz
Seoul National University Hospital — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Sclerosing cholangitis.
5 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Sclerosing cholangitis.
Community
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Start the conversation →Latest news about Sclerosing cholangitis
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What stage is my liver disease at, and how quickly might it progress?,How often do I need imaging and blood tests to monitor my bile ducts and liver?,Should I be screened for bile duct cancer, and how is that done?,Are there any clinical trials I might be eligible for?,Do I need to be evaluated for a liver transplant now or in the future?,What symptoms should prompt me to go to the emergency room immediately?,Should I be tested for inflammatory bowel disease if I haven't been already?
Common questions about Sclerosing cholangitis
What is Sclerosing cholangitis?
Sclerosing cholangitis is a serious liver disease that affects the bile ducts — the tubes that carry bile from the liver to the small intestine. In this condition, the bile ducts become inflamed and scarred over time, causing them to narrow and eventually block the flow of bile. When bile cannot drain properly, it builds up in the liver and causes damage. There are two main forms: primary sclerosing cholangitis (PSC), which is the most common type and is often linked to inflammatory bowel disease, and secondary sclerosing cholangitis, which is caused by a known trigger such as infection, surge
How is Sclerosing cholangitis inherited?
Sclerosing cholangitis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Sclerosing cholangitis?
Yes — 5 recruiting clinical trials are currently listed for Sclerosing cholangitis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Sclerosing cholangitis?
25 specialists and care centers treating Sclerosing cholangitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.