Overview
Secondary sclerosing cholangitis (SSC) is a serious liver disease that causes scarring and narrowing of the bile ducts — the tubes that carry bile from the liver to the small intestine. Unlike primary sclerosing cholangitis, which has no clear cause, secondary sclerosing cholangitis always develops as a result of another condition or event that damages the bile ducts. Common triggers include long-term blockage of the bile ducts (such as from gallstones or a tumor), repeated infections of the bile ducts, certain medications, or complications from major surgery or critical illness. One well-recognized form is called sclerosing cholangitis in critically ill patients (SC-CIP), which can develop after a person has been on a ventilator or in intensive care for a long time. As the bile ducts become scarred and narrowed, bile builds up in the liver, causing inflammation and progressive liver damage. Over time, this can lead to cirrhosis — permanent scarring of the liver — and liver failure. Key symptoms include yellowing of the skin and eyes (jaundice), severe itching, abdominal pain especially in the upper right side, fatigue, fever, and dark urine. Some people also lose weight and feel generally unwell. Treatment focuses on removing or treating the underlying cause whenever possible. This might mean clearing a bile duct blockage, treating an infection with antibiotics, or stopping a harmful medication. Procedures to open narrowed bile ducts (such as endoscopic or surgical interventions) are often used. In advanced cases where the liver is severely damaged, a liver transplant may be the only life-saving option. Early diagnosis and treatment of the root cause are critical to slowing or stopping the disease.
Key symptoms:
Yellowing of the skin and whites of the eyes (jaundice)Intense itching all over the bodyPain or discomfort in the upper right side of the abdomenFatigue and low energyFever and chills (often from bile duct infections)Dark brown or tea-colored urinePale or clay-colored stoolsNausea and loss of appetiteUnintended weight lossSwelling of the abdomen due to fluid buildup (ascites)Easy bruising or bleedingConfusion or difficulty thinking clearly (in advanced liver disease)
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Secondary sclerosing cholangitis.
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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 Secondary sclerosing cholangitis.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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 is the most likely cause of my secondary sclerosing cholangitis, and can it be treated or removed?,How advanced is the damage to my bile ducts and liver right now?,What procedures or treatments do you recommend, and what are the risks and benefits?,Should I be evaluated for a liver transplant, and what would that process involve?,What symptoms should prompt me to go to the emergency room immediately?,Are there any dietary changes or supplements I should take to protect my liver?,How often do I need follow-up appointments and imaging to monitor my condition?
Common questions about Secondary sclerosing cholangitis
What is Secondary sclerosing cholangitis?
Secondary sclerosing cholangitis (SSC) is a serious liver disease that causes scarring and narrowing of the bile ducts — the tubes that carry bile from the liver to the small intestine. Unlike primary sclerosing cholangitis, which has no clear cause, secondary sclerosing cholangitis always develops as a result of another condition or event that damages the bile ducts. Common triggers include long-term blockage of the bile ducts (such as from gallstones or a tumor), repeated infections of the bile ducts, certain medications, or complications from major surgery or critical illness. One well-reco
How is Secondary sclerosing cholangitis inherited?
Secondary sclerosing cholangitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Secondary sclerosing cholangitis?
1 specialists and care centers treating Secondary sclerosing cholangitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.