Benign recurrent intrahepatic cholestasis

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ORPHA:65682OMIM:243300K83.1
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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
Hepatocellular carcinomaHP:0001402Cholestatic liver diseaseHP:0002611Acholic stoolsHP:0011985
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Benign recurrent intrahepatic cholestasis.

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No actively recruiting trials found for Benign recurrent intrahepatic cholestasis at this time.

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No specialists are currently listed for Benign recurrent intrahepatic cholestasis.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA 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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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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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.