Overview
Benign recurrent intrahepatic cholestasis, often called BRIC, is a rare inherited liver condition where bile — a digestive fluid made by the liver — temporarily stops flowing properly. This causes bile to build up inside the liver, leading to episodes of intense itching and yellowing of the skin and eyes (jaundice). The word 'benign' means that, unlike some other cholestasis conditions, BRIC does not usually cause permanent liver damage or scarring over time. The word 'recurrent' means these episodes come and go throughout a person's life. During an episode, people may feel extremely itchy all over their body, lose their appetite, feel tired, and notice their skin and eyes turning yellow. Episodes can last anywhere from a few weeks to several months, and then symptoms go away completely until the next episode. The time between episodes varies widely — some people go years without problems, while others have more frequent flares. Triggers can include infections, surgery, pregnancy, or hormonal changes, though often no clear trigger is found. There is no cure for BRIC, but treatments can help manage symptoms during episodes. These include medications to relieve itching, such as cholestyramine or rifampicin, and in severe cases, a procedure called nasobiliary drainage or plasmapheresis may be used. Most people with BRIC live a normal lifespan, though the unpredictable nature of episodes can significantly affect quality of life.
Also known as:
Key symptoms:
Intense, widespread itching all over the bodyYellowing of the skin and whites of the eyes (jaundice)Dark-colored urinePale or light-colored stoolsLoss of appetiteNauseaFatigue and low energyWeight loss during episodesAbdominal discomfortElevated liver enzymes on blood tests during episodes
Clinical phenotype terms (16)— hover any for plain English
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Benign recurrent intrahepatic cholestasis.
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Specialists
View all specialists →No specialists are currently listed for Benign recurrent intrahepatic cholestasis.
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 Benign recurrent intrahepatic cholestasis.
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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.Which type of BRIC do I have — type 1 (ATP8B1) or type 2 (ABCB11) — and does that change my treatment options?,What is the best medication to manage my itching during an episode, and when should I start taking it?,Are there any known triggers I should try to avoid to reduce the frequency of episodes?,Should other family members be tested for this condition?,What signs should prompt me to go to the emergency room during an episode?,Is there any risk that my condition could progress to a more serious liver disease over time?,If I am planning a pregnancy, what extra monitoring or precautions do I need?
Common questions about Benign recurrent intrahepatic cholestasis
What is Benign recurrent intrahepatic cholestasis?
Benign recurrent intrahepatic cholestasis, often called BRIC, is a rare inherited liver condition where bile — a digestive fluid made by the liver — temporarily stops flowing properly. This causes bile to build up inside the liver, leading to episodes of intense itching and yellowing of the skin and eyes (jaundice). The word 'benign' means that, unlike some other cholestasis conditions, BRIC does not usually cause permanent liver damage or scarring over time. The word 'recurrent' means these episodes come and go throughout a person's life. During an episode, people may feel extremely itchy al
How is Benign recurrent intrahepatic cholestasis inherited?
Benign recurrent intrahepatic cholestasis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.