Overview
IgG4-related submandibular gland disease is a condition where the immune system causes chronic inflammation and swelling of the submandibular glands, which are the saliva-producing glands located beneath the jaw on both sides. This disease is part of a broader group of conditions called IgG4-related disease (IgG4-RD), in which a specific type of antibody called immunoglobulin G4 (IgG4) is found at unusually high levels in the blood and in affected tissues. When this condition targets the submandibular glands, it was historically known as Küttner tumor or chronic sclerosing sialadenitis. Patients typically notice painless, firm swelling under the jaw that may affect one or both sides. The swelling can come and go or gradually worsen over time. Because the glands become scarred and damaged, saliva production may decrease, leading to dry mouth. Some patients also develop swelling in other glands or organs, since IgG4-related disease can affect multiple parts of the body including the pancreas, tear glands, lymph nodes, and kidneys. Treatment usually begins with corticosteroids (such as prednisone), which are often very effective at reducing swelling and inflammation. For patients who relapse or cannot tolerate steroids, other immune-suppressing medications like rituximab or azathioprine may be used. In some cases, surgical removal of the affected gland is considered. Early diagnosis and treatment are important to prevent permanent gland damage from scarring.
Also known as:
Key symptoms:
Painless firm swelling under the jawSwelling on one or both sides of the jawDry mouth due to reduced saliva productionDifficulty eating dry foodsSwollen lymph nodes near the jaw or neckFatigueSwelling of other glands such as tear glands or parotid glandsWeight loss in some casesMild discomfort or tenderness in the swollen areaRecurrent episodes of gland swelling
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 submandibular gland disease.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for IgG4-related submandibular gland disease at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for IgG4-related submandibular gland disease.
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 submandibular gland disease.
Community
No community posts yet. Be the first to share your experience with IgG4-related submandibular gland disease.
Start the conversation →Latest news about IgG4-related submandibular gland disease
No recent news articles for IgG4-related submandibular gland disease.
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 submandibular gland, or are other organs affected?,What are my treatment options, and what are the risks and benefits of each?,How long will I need to take corticosteroids, and how will we manage side effects?,What is the likelihood that my disease will relapse after treatment?,Should I be screened for involvement of other organs like the pancreas or kidneys?,Are there clinical trials available for IgG4-related disease that I might be eligible for?,What can I do to manage dry mouth and protect my dental health?
Common questions about IgG4-related submandibular gland disease
What is IgG4-related submandibular gland disease?
IgG4-related submandibular gland disease is a condition where the immune system causes chronic inflammation and swelling of the submandibular glands, which are the saliva-producing glands located beneath the jaw on both sides. This disease is part of a broader group of conditions called IgG4-related disease (IgG4-RD), in which a specific type of antibody called immunoglobulin G4 (IgG4) is found at unusually high levels in the blood and in affected tissues. When this condition targets the submandibular glands, it was historically known as Küttner tumor or chronic sclerosing sialadenitis. Patie
How is IgG4-related submandibular gland disease inherited?
IgG4-related submandibular gland disease follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related submandibular gland disease typically begin?
Typical onset of IgG4-related submandibular gland disease is adult. Age of onset can vary across affected individuals.