Overview
Insulin autoimmune syndrome (IAS), also known as Hirata disease, is a rare condition where your body makes antibodies that attack your own insulin. These antibodies bind to insulin in your blood and then release it at unpredictable times, causing episodes of dangerously low blood sugar (hypoglycemia). This typically happens several hours after eating, when the antibodies suddenly let go of stored insulin all at once, flooding your body with too much active insulin. The condition was first described by Dr. Yukimasa Hirata in Japan in 1970. Unlike other causes of low blood sugar, people with IAS have never taken insulin injections. Blood tests show very high levels of insulin along with antibodies directed against insulin. The syndrome can develop on its own or may be triggered by certain medications, particularly those containing a chemical group called a sulfhydryl group (such as methimazole, used for overactive thyroid). Many cases of IAS resolve on their own, especially if a triggering medication is stopped. Treatment focuses on preventing low blood sugar episodes through dietary changes, such as eating small frequent meals. In more stubborn cases, medications like diazoxide, corticosteroids, or other immune-suppressing drugs may be used. In rare severe cases, surgery to remove part of the pancreas has been considered, though this is generally avoided.
Also known as:
Key symptoms:
Episodes of very low blood sugar (hypoglycemia)Shakiness and tremblingSweating, especially cold sweatsConfusion or difficulty thinking clearlyBlurred visionExtreme hungerHeart pounding or racing heartbeatDizziness or lightheadednessWeakness and fatigueAnxiety or nervousnessSeizures in severe casesLoss of consciousness in severe casesSymptoms typically occurring a few hours after meals
Clinical phenotype terms (13)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Insulin autoimmune syndrome.
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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 Insulin autoimmune syndrome.
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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.Could any of my current medications be triggering this condition?,How often should I check my blood sugar, and what levels should concern me?,What dietary changes will help prevent low blood sugar episodes?,Is this condition likely to resolve on its own, and if so, how long might that take?,Should I carry a glucagon emergency kit, and how do I use it?,Are there any medications or over-the-counter drugs I should avoid?,Should my family members be tested for HLA types or insulin antibodies?
Common questions about Insulin autoimmune syndrome
What is Insulin autoimmune syndrome?
Insulin autoimmune syndrome (IAS), also known as Hirata disease, is a rare condition where your body makes antibodies that attack your own insulin. These antibodies bind to insulin in your blood and then release it at unpredictable times, causing episodes of dangerously low blood sugar (hypoglycemia). This typically happens several hours after eating, when the antibodies suddenly let go of stored insulin all at once, flooding your body with too much active insulin. The condition was first described by Dr. Yukimasa Hirata in Japan in 1970. Unlike other causes of low blood sugar, people with IA
How is Insulin autoimmune syndrome inherited?
Insulin autoimmune syndrome follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Insulin autoimmune syndrome typically begin?
Typical onset of Insulin autoimmune syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Insulin autoimmune syndrome?
7 specialists and care centers treating Insulin autoimmune syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.