Overview
Follicular cholangitis and pancreatitis is a very rare condition that affects the bile ducts (the tubes that carry bile from the liver) and the pancreas. It is characterized by inflammation in these areas that has a distinctive pattern under the microscope, where clusters of immune cells called lymphoid follicles form around the bile ducts and pancreatic ducts. This condition is sometimes considered part of the spectrum of IgG4-related disease or autoimmune pancreatitis, but it has unique features that set it apart. Patients may experience abdominal pain, jaundice (yellowing of the skin and eyes), itching, weight loss, and digestive problems. The inflammation can cause narrowing or blockage of the bile ducts, which may mimic bile duct cancer or other serious conditions. Diagnosis often requires tissue biopsy to see the characteristic follicular pattern of inflammation. Treatment typically involves corticosteroids (such as prednisone) to reduce inflammation, and in some cases, procedures to relieve bile duct obstruction. Because this condition is so rare, management is often guided by experience with related autoimmune biliary and pancreatic diseases. Early recognition is important to avoid unnecessary surgery that might be performed if the condition is mistaken for cancer.
Also known as:
Key symptoms:
Abdominal pain, especially in the upper bellyYellowing of the skin and eyes (jaundice)Itchy skinUnexplained weight lossNausea or vomitingPale or clay-colored stoolsDark urineFatigue and tirednessLoss of appetiteDigestive problems such as diarrhea or fatty stoolsFever in some casesSwelling or mass in the pancreas area
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
1 availableAzithromycin
Urethritis and cervicitis in adults
Clinical Trials
View all trials with filters →No actively recruiting trials found for Follicular cholangitis and pancreatitis at this time.
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Specialists
View all specialists →No specialists are currently listed for Follicular cholangitis and pancreatitis.
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 Follicular cholangitis and pancreatitis.
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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.How certain is the diagnosis, and could this be something else like cancer or IgG4-related disease?,What treatment do you recommend, and how long will I need to take steroids?,What are the side effects of the medications, and how will they be monitored?,Will I need any procedures to open up blocked bile ducts?,How often will I need follow-up imaging or blood tests?,What symptoms should prompt me to seek emergency care?,Is there a risk of this condition coming back after treatment?
Common questions about Follicular cholangitis and pancreatitis
What is Follicular cholangitis and pancreatitis?
Follicular cholangitis and pancreatitis is a very rare condition that affects the bile ducts (the tubes that carry bile from the liver) and the pancreas. It is characterized by inflammation in these areas that has a distinctive pattern under the microscope, where clusters of immune cells called lymphoid follicles form around the bile ducts and pancreatic ducts. This condition is sometimes considered part of the spectrum of IgG4-related disease or autoimmune pancreatitis, but it has unique features that set it apart. Patients may experience abdominal pain, jaundice (yellowing of the skin and ey
How is Follicular cholangitis and pancreatitis inherited?
Follicular cholangitis and pancreatitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Follicular cholangitis and pancreatitis typically begin?
Typical onset of Follicular cholangitis and pancreatitis is adult. Age of onset can vary across affected individuals.