Overview
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare inflammatory disease that affects the bile ducts — the tubes that carry bile from the liver to the small intestine. It is part of a larger group of conditions called IgG4-related disease (IgG4-RD), where the immune system produces too many of a certain type of antibody called IgG4, leading to inflammation and scarring (fibrosis) in various organs. In IgG4-SC, this process mainly targets the bile ducts, causing them to narrow and become blocked over time. The most common symptoms include yellowing of the skin and eyes (jaundice), dark urine, pale stools, itching, and abdominal pain. Many people also experience weight loss and fatigue. Because the bile ducts are closely connected to the pancreas, IgG4-SC often occurs alongside a condition called autoimmune pancreatitis (AIP), and the two are frequently diagnosed together. The disease is sometimes mistaken for bile duct cancer or primary sclerosing cholangitis (PSC), which can delay diagnosis. The good news is that IgG4-SC usually responds very well to treatment with corticosteroids, particularly prednisone. Most patients see significant improvement after starting steroids. However, relapses are common, and some people need long-term treatment to keep the disease under control. Other medications such as rituximab or azathioprine may be used to help maintain remission and reduce steroid side effects. Early diagnosis and treatment are important to prevent permanent scarring of the bile ducts and liver damage.
Key symptoms:
Yellowing of the skin and whites of the eyes (jaundice)Dark, tea-colored urinePale or clay-colored stoolsItchy skinUpper abdominal pain or discomfortUnexplained weight lossFatigue and low energyNauseaFever (less common)Swelling or tenderness in the abdomenSigns of liver problems such as elevated liver enzymes on blood tests
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
1 availableWEGOVY
To reduce excess body weight and maintain weight reduction long term in adults with overweight in the presence of at least one weight-related comorbid condition
Clinical Trials
View all trials with filters →No actively recruiting trials found for IgG4-related sclerosing cholangitis at this time.
New trials open frequently. Follow this disease to get notified.
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
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to IgG4-related sclerosing cholangitis.
Community
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Start the conversation →Latest news about IgG4-related sclerosing cholangitis
1 articlesCaregiver 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.How confident are you in the diagnosis of IgG4-SC, and have other conditions like bile duct cancer or primary sclerosing cholangitis been ruled out?,What organs besides my bile ducts are affected, and do I need to be checked for IgG4-related disease elsewhere in my body?,What is my treatment plan, and how long will I need to take steroids?,What are the signs that my disease is relapsing, and what should I do if I notice them?,Are there steroid-sparing medications I should consider to reduce long-term steroid side effects?,How often will I need follow-up appointments and imaging to monitor my bile ducts and liver?,Is there a clinical trial or specialist center for IgG4-related disease that I should be referred to?
Common questions about IgG4-related sclerosing cholangitis
What is IgG4-related sclerosing cholangitis?
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare inflammatory disease that affects the bile ducts — the tubes that carry bile from the liver to the small intestine. It is part of a larger group of conditions called IgG4-related disease (IgG4-RD), where the immune system produces too many of a certain type of antibody called IgG4, leading to inflammation and scarring (fibrosis) in various organs. In IgG4-SC, this process mainly targets the bile ducts, causing them to narrow and become blocked over time. The most common symptoms include yellowing of the skin and eyes (jaundice), dark uri
How is IgG4-related sclerosing cholangitis inherited?
IgG4-related sclerosing cholangitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related sclerosing cholangitis typically begin?
Typical onset of IgG4-related sclerosing cholangitis is adult. Age of onset can vary across affected individuals.
Which specialists treat IgG4-related sclerosing cholangitis?
25 specialists and care centers treating IgG4-related sclerosing cholangitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for IgG4-related sclerosing cholangitis?
1 patient support program are currently tracked on UniteRare for IgG4-related sclerosing cholangitis. See the treatments and support programs sections for copay assistance, eligibility, and contact details.