Overview
IgG4-related mediastinitis is a rare inflammatory disease that affects the mediastinum — the space in the middle of the chest between the lungs that contains the heart, major blood vessels, windpipe, and esophagus. It is part of a larger group of conditions called IgG4-related disease (IgG4-RD), where the immune system produces too many of a specific type of antibody called IgG4, leading to inflammation and the buildup of scar-like tissue (fibrosis) in various organs. When this process happens in the mediastinum, it can compress or block important structures in the chest. This condition is also sometimes called IgG4-related sclerosing mediastinitis or fibrosing mediastinitis due to IgG4-RD. The disease can cause a wide range of symptoms depending on which structures in the chest are being compressed. Common problems include chest pain, shortness of breath, difficulty swallowing, and swelling of the face, neck, or arms if major veins are blocked. Some people may have no symptoms at first, and the disease is discovered by accident on a chest scan done for another reason. Treatment usually involves medicines that calm the immune system, most commonly steroids like prednisone. Other drugs that suppress the immune system, such as rituximab, may also be used, especially if steroids alone are not enough or if the disease comes back. With proper treatment, many people improve significantly, though some may need long-term therapy to prevent relapse. Early diagnosis and treatment are important to prevent permanent scarring and serious complications.
Key symptoms:
Chest pain or pressureShortness of breathDifficulty swallowingSwelling of the face, neck, or arms (from blocked veins)Persistent coughHoarse voiceFatigueUnexplained weight lossFever (less common)Feeling of fullness or tightness in the chest
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventBeijing Chao Yang Hospital — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for IgG4-related mediastinitis.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for IgG4-related mediastinitis.
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 mediastinitis.
Community
No community posts yet. Be the first to share your experience with IgG4-related mediastinitis.
Start the conversation →Latest news about IgG4-related mediastinitis
Disease timeline:
New recruiting trial: Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension
A new clinical trial is recruiting patients for IgG4-related mediastinitis
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 certain are you of the diagnosis, and do I need a biopsy to confirm it?,Which organs besides my chest might be affected, and how will you check for that?,What is the treatment plan, and how long will I need to take steroids?,What are the signs that the disease is coming back, and what should I do if that happens?,Are there clinical trials for IgG4-related disease that I might be eligible for?,What long-term monitoring will I need, and how often should I have imaging or blood tests?,Should I see any other specialists, such as a rheumatologist or pulmonologist?
Common questions about IgG4-related mediastinitis
What is IgG4-related mediastinitis?
IgG4-related mediastinitis is a rare inflammatory disease that affects the mediastinum — the space in the middle of the chest between the lungs that contains the heart, major blood vessels, windpipe, and esophagus. It is part of a larger group of conditions called IgG4-related disease (IgG4-RD), where the immune system produces too many of a specific type of antibody called IgG4, leading to inflammation and the buildup of scar-like tissue (fibrosis) in various organs. When this process happens in the mediastinum, it can compress or block important structures in the chest. This condition is als
How is IgG4-related mediastinitis inherited?
IgG4-related mediastinitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related mediastinitis typically begin?
Typical onset of IgG4-related mediastinitis is adult. Age of onset can vary across affected individuals.
Are there clinical trials for IgG4-related mediastinitis?
Yes — 1 recruiting clinical trial is currently listed for IgG4-related mediastinitis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.