Overview
Insulin-resistance syndrome type B, also known as type B insulin resistance, is a rare autoimmune condition in which the body produces antibodies that attack the insulin receptors on cells. Insulin is a hormone that helps your cells take in sugar (glucose) from the blood for energy. When antibodies block or interfere with insulin receptors, your cells cannot respond properly to insulin, leading to very high blood sugar levels that are often extremely difficult to control. In some cases, the antibodies can actually stimulate the insulin receptor, causing dangerously low blood sugar (hypoglycemia) instead. This condition most commonly affects adult women, and it is frequently associated with other autoimmune disorders such as systemic lupus erythematosus (SLE or lupus). Patients often develop a dark, velvety skin thickening called acanthosis nigricans, especially in skin folds like the neck, armpits, and groin. Severe hyperglycemia (high blood sugar) that does not respond well to even very large doses of insulin is a hallmark feature. Some patients also experience significant weight loss. Treatment focuses on suppressing the immune system to reduce the production of the harmful antibodies. Doctors may use medications such as corticosteroids, rituximab, cyclophosphamide, or plasmapheresis (a procedure that filters antibodies from the blood). In many cases, the condition can go into remission with aggressive immunosuppressive therapy, though it may take months. Managing blood sugar levels during active disease can be very challenging and often requires close monitoring in a specialized medical center.
Key symptoms:
Very high blood sugar that does not respond to insulin injectionsDark, velvety skin patches (acanthosis nigricans), especially on the neck and armpitsUnexplained weight lossExtreme thirst and frequent urinationEpisodes of dangerously low blood sugar in some casesFatigue and weaknessBlurred vision from high blood sugarJoint pain or other signs of autoimmune diseaseIncreased appetite despite weight lossSkin infections or slow wound healingEnlarged ovaries or menstrual irregularities in women
Clinical phenotype terms (50)— 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 Insulin-resistance syndrome type B.
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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-resistance syndrome type B.
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Questions for your doctor
Bring these to your next appointment
- Q1.What is my current antibody level, and how will you track whether treatment is working?,Which immunosuppressive treatment do you recommend, and what are the side effects I should watch for?,How should I manage my blood sugar at home when it is not responding to insulin?,What are the warning signs that I should go to the emergency room?,Is there a risk that I could switch from high blood sugar to low blood sugar during treatment?,How long does treatment usually take before I might see improvement?,Should I be screened for other autoimmune conditions like lupus?
Common questions about Insulin-resistance syndrome type B
What is Insulin-resistance syndrome type B?
Insulin-resistance syndrome type B, also known as type B insulin resistance, is a rare autoimmune condition in which the body produces antibodies that attack the insulin receptors on cells. Insulin is a hormone that helps your cells take in sugar (glucose) from the blood for energy. When antibodies block or interfere with insulin receptors, your cells cannot respond properly to insulin, leading to very high blood sugar levels that are often extremely difficult to control. In some cases, the antibodies can actually stimulate the insulin receptor, causing dangerously low blood sugar (hypoglycemi
How is Insulin-resistance syndrome type B inherited?
Insulin-resistance syndrome type B follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Insulin-resistance syndrome type B typically begin?
Typical onset of Insulin-resistance syndrome type B is adult. Age of onset can vary across affected individuals.
Which specialists treat Insulin-resistance syndrome type B?
5 specialists and care centers treating Insulin-resistance syndrome type B are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.