Type 1 interferonopathy

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Type 1 interferonopathy is a group of rare inherited immune system disorders. The name comes from 'interferon,' a protein your body normally makes to fight viruses. In people with this condition, the immune system acts as if it is constantly fighting a virus — even when there is no infection. This leads to ongoing inflammation that can damage many parts of the body over time. These conditions are caused by changes (mutations) in specific genes that control how the immune system responds to threats. When these genes do not work properly, the body produces too much type 1 interferon, which keeps the immune system in a constant state of alarm. This can affect the brain, skin, joints, blood vessels, lungs, and other organs. Common symptoms include skin rashes (especially on the face, hands, and feet), joint pain, brain problems such as seizures or developmental delays, and recurring fevers. Some people also develop chilblain-like sores on their fingers and toes. Treatment usually focuses on calming the overactive immune system using medications such as JAK inhibitors (for example, baricitinib or ruxolitinib), which have shown promise in reducing symptoms. There is currently no cure, but early treatment can help slow damage and improve quality of life. This group of diseases is also sometimes called 'type I interferonopathy' or 'interferonopathy type 1.'

Key symptoms:

Skin rashes, especially on the face, ears, hands, and feetChilblain-like sores (painful, red or purple patches on fingers and toes triggered by cold)Joint pain and swellingRecurring fevers without a clear infectionSeizuresDevelopmental delays or intellectual disabilityMuscle stiffness or weaknessCalcium deposits in the brain (calcifications)Lung problems such as shortness of breathEnlarged liver or spleenAnemia (low red blood cell count)Eye inflammationFatigue and low energySlow head growth in infants (microcephaly)Blood vessel inflammation (vasculopathy)

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Jan 2026Phase 1b Safety Study of IMSB301 in Type 1 Interferonopathies

ImmuneSensor Therapeutics Inc. — PHASE1

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Type 1 interferonopathy.

View clinical trials →

No actively recruiting trials found for Type 1 interferonopathy at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Type 1 interferonopathy community →

No specialists are currently listed for Type 1 interferonopathy.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Type 1 interferonopathy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Type 1 interferonopathy

Disease timeline:

New recruiting trial: Phase 1b Safety Study of IMSB301 in Type 1 Interferonopathies

A new clinical trial is recruiting patients for Type 1 interferonopathy

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.Which specific subtype of type 1 interferonopathy does my child or I have, and what does that mean for our outlook?,Should we do genetic testing for other family members, and what are the chances of passing this on to children?,Is my child or am I a candidate for JAK inhibitor treatment, and what are the risks and benefits?,How often should we have brain imaging or other monitoring tests?,Are there any clinical trials we should consider joining?,What signs should prompt us to go to the emergency room?,Are there any lifestyle changes — such as avoiding cold or certain activities — that could help manage symptoms?

Common questions about Type 1 interferonopathy

What is Type 1 interferonopathy?

Type 1 interferonopathy is a group of rare inherited immune system disorders. The name comes from 'interferon,' a protein your body normally makes to fight viruses. In people with this condition, the immune system acts as if it is constantly fighting a virus — even when there is no infection. This leads to ongoing inflammation that can damage many parts of the body over time. These conditions are caused by changes (mutations) in specific genes that control how the immune system responds to threats. When these genes do not work properly, the body produces too much type 1 interferon, which keep