Overview
Autoinflammatory syndrome of childhood is a rare condition in which the body's immune system becomes overactive without an infection or other clear trigger. The term covers a group of disorders where the innate immune system — the body's first line of defense — fires up on its own, causing repeated episodes of inflammation. These flare-ups can affect many parts of the body, including the skin, joints, eyes, and internal organs. Children with this condition typically experience recurring fevers that come and go in patterns, along with skin rashes, joint pain and swelling, abdominal pain, and general feelings of being unwell. The episodes may last days to weeks and can vary in severity. Between flare-ups, children may feel completely normal or may have lingering low-grade symptoms. Treatment depends on the specific type of autoinflammatory syndrome involved. Many patients benefit from medications that block specific inflammatory pathways, such as interleukin-1 (IL-1) inhibitors like anakinra or canakinumab, or other biologic therapies. Anti-inflammatory drugs such as colchicine or corticosteroids may also be used. Early diagnosis and treatment are important to prevent long-term complications like amyloidosis, which can damage the kidneys and other organs. Management usually requires a team of specialists working together to control inflammation and improve quality of life.
Key symptoms:
Recurring fevers that come and goSkin rashes during flare-upsJoint pain and swellingAbdominal painMouth sores or ulcersEye redness or inflammationSwollen lymph nodesFatigue and feeling unwell during episodesHeadachesMuscle achesChest pain from inflammation of the lining around the lungs or heartGrowth delays in some childrenElevated inflammatory markers in blood tests
Variable
Can be inherited in different ways depending on the underlying gene
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Autoinflammatory syndrome of childhood.
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Specialists
View all specialists →No specialists are currently listed for Autoinflammatory syndrome of childhood.
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 Autoinflammatory syndrome of childhood.
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Caregiver Resources
NORD Caregiver Resources
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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.What specific type of autoinflammatory syndrome does my child have, and what gene is involved?,What medications are recommended, and what are the potential side effects?,How will we monitor for complications like amyloidosis or organ damage?,What should I do when my child has a flare-up — when should I call the doctor versus go to the emergency room?,Are there any vaccines my child should avoid while on immunosuppressive medications?,Will my child need to take medication for life, or could they outgrow this condition?,Should other family members be tested for this condition?
Common questions about Autoinflammatory syndrome of childhood
What is Autoinflammatory syndrome of childhood?
Autoinflammatory syndrome of childhood is a rare condition in which the body's immune system becomes overactive without an infection or other clear trigger. The term covers a group of disorders where the innate immune system — the body's first line of defense — fires up on its own, causing repeated episodes of inflammation. These flare-ups can affect many parts of the body, including the skin, joints, eyes, and internal organs. Children with this condition typically experience recurring fevers that come and go in patterns, along with skin rashes, joint pain and swelling, abdominal pain, and g
At what age does Autoinflammatory syndrome of childhood typically begin?
Typical onset of Autoinflammatory syndrome of childhood is childhood. Age of onset can vary across affected individuals.