Overview
IgG4-related aortitis is a rare inflammatory condition that affects the aorta, which is the largest blood vessel in your body. It is part of a broader group of conditions called IgG4-related disease (IgG4-RD), where a specific type of immune cell produces too much of an antibody called immunoglobulin G4 (IgG4). This leads to chronic inflammation and thickening of the aortic wall, which can weaken the vessel over time and potentially cause dangerous complications like aortic aneurysms (bulging of the vessel wall) or narrowing of the blood vessel. Many patients with IgG4-related aortitis may not notice symptoms at first. When symptoms do appear, they can include chest or back pain, fatigue, fever, weight loss, and general feelings of being unwell. Some patients develop symptoms related to reduced blood flow if the inflammation narrows or blocks nearby arteries. The condition can also affect other organs at the same time, including the pancreas, kidneys, salivary glands, and tissues around the eyes. Treatment typically involves medications that calm the immune system. Corticosteroids like prednisone are the first-line treatment and often produce a good response. For patients who relapse or cannot tolerate steroids, other immunosuppressive drugs or the biologic medication rituximab may be used. Surgery may be needed if an aneurysm develops. Early diagnosis and treatment are important to prevent serious complications like aortic rupture.
Key symptoms:
Chest painBack painAbdominal painUnexplained fatigueFeverUnintentional weight lossGeneral feeling of being unwellSwelling of the aorta (aortic aneurysm)Thickening of tissue around the aortaHigh blood pressureReduced blood flow to organsSwollen lymph nodesSymptoms from other affected organs such as swollen salivary glands or kidney problems
Clinical phenotype terms (25)— hover any for plain English
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 aortitis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for IgG4-related aortitis 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 aortitis.
Community
No community posts yet. Be the first to share your experience with IgG4-related aortitis.
Start the conversation →Latest news about IgG4-related aortitis
No recent news articles for IgG4-related aortitis.
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.How severe is the inflammation in my aorta, and has any permanent damage occurred?,Are any other organs affected by IgG4-related disease in my case?,What is the plan for tapering steroids, and what should I do if symptoms return?,Am I a candidate for rituximab or other steroid-sparing medications?,How often will I need imaging and blood tests to monitor my condition?,What are the warning signs of a serious complication that should send me to the emergency room?,Are there any clinical trials for IgG4-related disease that I might be eligible for?
Common questions about IgG4-related aortitis
What is IgG4-related aortitis?
IgG4-related aortitis is a rare inflammatory condition that affects the aorta, which is the largest blood vessel in your body. It is part of a broader group of conditions called IgG4-related disease (IgG4-RD), where a specific type of immune cell produces too much of an antibody called immunoglobulin G4 (IgG4). This leads to chronic inflammation and thickening of the aortic wall, which can weaken the vessel over time and potentially cause dangerous complications like aortic aneurysms (bulging of the vessel wall) or narrowing of the blood vessel. Many patients with IgG4-related aortitis may no
How is IgG4-related aortitis inherited?
IgG4-related aortitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related aortitis typically begin?
Typical onset of IgG4-related aortitis is adult. Age of onset can vary across affected individuals.
Which specialists treat IgG4-related aortitis?
16 specialists and care centers treating IgG4-related aortitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.