Overview
IgG4-related mesenteritis is a rare condition that falls under the broader group of IgG4-related diseases (IgG4-RD). In this condition, the mesentery — the tissue that connects your intestines to the wall of your abdomen and carries blood vessels, nerves, and lymph nodes — becomes inflamed and thickened due to an abnormal immune response. The hallmark of this disease is the infiltration of a specific type of immune cell that produces IgG4 antibodies, along with fibrosis (scarring) of the affected tissue. Patients may also hear this condition referred to as IgG4-related sclerosing mesenteritis. Symptoms can vary widely but often include abdominal pain, bloating, nausea, weight loss, and sometimes a palpable mass in the abdomen. Some patients may develop bowel obstruction if the inflammation and scarring become severe enough to compress the intestines. Because IgG4-related disease can affect multiple organs, patients may also have involvement of the pancreas, bile ducts, salivary glands, kidneys, or other tissues at the same time or at different points in their disease course. Treatment typically involves corticosteroids such as prednisone, which are often very effective at reducing inflammation and shrinking the affected tissue. For patients who relapse or cannot tolerate steroids, steroid-sparing immunosuppressive medications like azathioprine, mycophenolate mofetil, or rituximab (a biologic therapy) may be used. Early diagnosis and treatment are important to prevent irreversible fibrosis and organ damage.
Also known as:
Key symptoms:
Abdominal pain or discomfortAbdominal bloatingNausea or vomitingUnintended weight lossLoss of appetiteA lump or mass felt in the abdomenBowel obstruction symptoms such as constipation or inability to pass gasFatigueFever (occasionally)DiarrheaSwelling of lymph nodes in the abdomenBack painSymptoms from other organs affected by IgG4-related disease (e.g., jaundice, dry mouth)
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for IgG4-related mesenteritis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for IgG4-related mesenteritis 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
Travel Grants
No travel grants are currently matched to IgG4-related mesenteritis.
Community
No community posts yet. Be the first to share your experience with IgG4-related mesenteritis.
Start the conversation →Latest news about IgG4-related mesenteritis
No recent news articles for IgG4-related mesenteritis.
Follow this condition to be notified when news becomes available.
Caregiver 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.Is my disease limited to the mesentery, or are other organs also affected?,What are the risks and benefits of starting steroid treatment, and how long will I need to take it?,What is the plan if my disease comes back after treatment?,How often will I need blood tests and imaging to monitor my condition?,Should I be screened for IgG4-related disease in other organs?,Are there clinical trials or newer treatments I should consider?,What symptoms should prompt me to seek emergency care?
Common questions about IgG4-related mesenteritis
What is IgG4-related mesenteritis?
IgG4-related mesenteritis is a rare condition that falls under the broader group of IgG4-related diseases (IgG4-RD). In this condition, the mesentery — the tissue that connects your intestines to the wall of your abdomen and carries blood vessels, nerves, and lymph nodes — becomes inflamed and thickened due to an abnormal immune response. The hallmark of this disease is the infiltration of a specific type of immune cell that produces IgG4 antibodies, along with fibrosis (scarring) of the affected tissue. Patients may also hear this condition referred to as IgG4-related sclerosing mesenteritis.
How is IgG4-related mesenteritis inherited?
IgG4-related mesenteritis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related mesenteritis typically begin?
Typical onset of IgG4-related mesenteritis is adult. Age of onset can vary across affected individuals.
Which specialists treat IgG4-related mesenteritis?
3 specialists and care centers treating IgG4-related mesenteritis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.