Overview
IgG4-related dacryoadenitis and sialadenitis is a rare immune system condition that causes swelling and inflammation in the tear glands (dacryoadenitis) and the saliva-producing glands (sialadenitis). It belongs to a broader group of conditions called IgG4-related disease (IgG4-RD). In this condition, a type of immune protein called IgG4 builds up in the glands, causing them to become enlarged and inflamed over time. The disease can affect the glands around the eyes and in the cheeks, jaw, and under the tongue. It was previously known by other names, including Mikulicz disease and Küttner tumor, depending on which glands were affected. The most noticeable symptoms are painless swelling around the eyes and in the cheeks or jaw area. Some people experience dry eyes and dry mouth because the inflamed glands cannot produce enough tears or saliva. The swelling can look alarming but is usually not painful. Without treatment, the glands can become permanently damaged. The good news is that this condition often responds very well to treatment. Corticosteroids like prednisone are the main treatment and can reduce swelling quickly. Some people need additional medications to prevent the disease from coming back. With proper care, many patients do very well and can lead normal lives.
Also known as:
Key symptoms:
Painless swelling around one or both eyesSwelling of the cheeks or jaw area due to enlarged saliva glandsDry eyes or a gritty feeling in the eyesDry mouth and difficulty swallowingReduced ability to produce tearsEnlarged lymph nodes in the neckMild fatigueOccasional mild discomfort or pressure around the eyes or faceDifficulty wearing contact lenses due to dry eyesRecurrent swelling that comes and goes over time
Clinical phenotype terms (33)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventREBETOL: FDA approved
Treatment of chronic hepatitis C among previously untreated pediatric patients at least three years of age or older.
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableREBETOL
Treatment of chronic hepatitis C among previously untreated pediatric patients at least three years of age or older.
Clinical Trials
View all trials with filters →No actively recruiting trials found for IgG4-related dacryoadenitis and sialadenitis 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 dacryoadenitis and sialadenitis.
Community
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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 my diagnosis, and do I need a biopsy to confirm it?,What is my IgG4 blood level, and what does it mean for my treatment?,How long will I need to take prednisone, and what are the risks of long-term use?,What are the signs that my disease is coming back, and what should I do if that happens?,Are there other organs in my body that could be affected by IgG4-related disease?,Should I see other specialists such as an eye doctor or a kidney specialist?,Are there any clinical trials I might be eligible for?
Common questions about IgG4-related dacryoadenitis and sialadenitis
What is IgG4-related dacryoadenitis and sialadenitis?
IgG4-related dacryoadenitis and sialadenitis is a rare immune system condition that causes swelling and inflammation in the tear glands (dacryoadenitis) and the saliva-producing glands (sialadenitis). It belongs to a broader group of conditions called IgG4-related disease (IgG4-RD). In this condition, a type of immune protein called IgG4 builds up in the glands, causing them to become enlarged and inflamed over time. The disease can affect the glands around the eyes and in the cheeks, jaw, and under the tongue. It was previously known by other names, including Mikulicz disease and Küttner tumo
How is IgG4-related dacryoadenitis and sialadenitis inherited?
IgG4-related dacryoadenitis and sialadenitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related dacryoadenitis and sialadenitis typically begin?
Typical onset of IgG4-related dacryoadenitis and sialadenitis is adult. Age of onset can vary across affected individuals.
Which specialists treat IgG4-related dacryoadenitis and sialadenitis?
15 specialists and care centers treating IgG4-related dacryoadenitis and sialadenitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for IgG4-related dacryoadenitis and sialadenitis?
1 patient support program are currently tracked on UniteRare for IgG4-related dacryoadenitis and sialadenitis. See the treatments and support programs sections for copay assistance, eligibility, and contact details.