National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Insulin autoimmune syndrome


Other Names:
Hirata disease; Insulin autoimmune hypoglycemia
Insulin autoimmune syndrome is a rare condition that causes low blood sugar (hypoglycemia). This occurs because the body begins to make a specific kind of protein called antibodies to attack insulin. Insulin is a naturally occurring hormone that is responsible for keeping blood sugar at a normal level. When blood sugar levels get too high, insulin helps to store the sugar for future use. People affected by insulin autoimmune syndrome have antibodies that attack insulin, causing it to work too hard and the level of blood sugar to become too low. Insulin autoimmune syndrome most often begins during adulthood.[1][2]
Last updated: 11/13/2016
The symptoms of insulin autoimmune syndrome are similar to those of other disorders that cause low blood sugar. These symptoms include sweating, fainting, lightheadedness, weakness, and fatigue. If a person has symptoms of insulin autoimmune syndrome for a long time, the high levels of insulin can begin to attack the pancreas, which can cause damage to the organ.[3][4]  
Last updated: 11/13/2016
Insulin autoimmune syndrome occurs suddenly as antibodies are produced that attack the insulin which the body is producing naturally. Because a person affected by this condition is producing antibodies to attack a naturally occurring substance, the condition is classified as a type of autoimmune syndrome. This means that the immune system, which is responsible for protecting the body from disease, is instead attacking the body itself.[1]

Exactly why these antibodies against insulin are suddenly produced can vary from person to person. In some cases, insulin autoimmune syndrome occurs in a person with another autoimmune disease, which can indicate that the immune system in the body is not behaving correctly. Additionally, a certain class of medications called sulphydryl compounds have been shown to sometimes cause insulin autoimmune syndrome. Some of these medications are used to treat other autoimmune conditions. Recently, a compound called alpha-lipoic acid has been associated with an increased risk of developing insulin autoimmune syndrome. This compound is sometimes used for dieting purposes.[5] 
Last updated: 11/13/2016
Insulin autoimmune syndrome may be diagnosed by measuring blood sugar levels, insulin levels, and ruling out other conditions that can cause low blood sugar. Certain tests may also be used to test for the presence of antibodies that attack insulin. All of these tests can be completed using blood from a blood test.[6] 
Last updated: 11/13/2016
The treatment of insulin autoimmune syndrome is similar to treatments for other conditions that cause low blood sugar. This includes eating smaller meals more frequently in order to maintain a more consistent blood sugar level and continuous monitoring of blood sugar. Additional treatment options can include treatment with steroids and a procedure called plasmaphoresis which removes the blood from the body, separates the insulin antibodies in the blood stream, and replaces the blood without the antibodies. Any damage to the pancreas may also require treatment.[7][8] 
Last updated: 11/13/2016
Most people affected by insulin autoimmune syndrome find that their symptoms go away after a few months. It may be important to stop taking any medications that may be triggering the condition. Some people, however, do experience recurrent attacks where they experience low blood sugar levels. With proper treatment, any recurrent episodes can likely be controlled.[2] 
Last updated: 11/13/2016

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • PubMed is a searchable database of medical literature and lists journal articles that discuss Insulin autoimmune syndrome. Click on the link to view a sample search on this topic.

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  1. Archambeaud-Mouveroux, F, Huc, MC, Nadalon, S, Fournier, MP, and B Canivet. Autoimmune insulin syndrome. Biomedicine & Pharmacotherapy. 1989; 43(8):581-586. https://www.ncbi.nlm.nih.gov/pubmed/2698754.
  2. Uchigata, Y, Eguchi, Y, Takayama-Hasumi, S, and Y Omori. Insulin autoimmune syndrome (Hirata Disease): clinical features and epidemiology in Japan. Diabetes Research and Clinical Practice. January 1994; 22(2-3):89-94. http://www.sciencedirect.com/science/article/pii/016882279490040X.
  3. M Funnell. Low Blood Glucose (Hypoglycemia). National Institute of Diabetes and Digestive and Kidney Diseases; August 2016; https://www.niddk.nih.gov/health-information/diabetes/preventing-diabetes-problems/low-blood-glucose-hypoglycemia.
  4. Bresciani, E, Bussi, A, Bazzigaluppi, E, and G Balestrieri. Insulin Autoimmune Syndrome Induced by a-Lipoic Acid in a Caucasian Woman: Case Report. Diabetes Care. September 2011; 34(9):e146-e146. http://care.diabetesjournals.org/content/34/9/e146.
  5. Uchigata, Y, Hirata, Y, and Y Iwamoto. Drug-induced insulin autoimmune syndrome. Diabetes Research and Clinical Practice. January 2009; 83(1):e19-20. https://www.ncbi.nlm.nih.gov/pubmed/19070385.
  6. Church, D, Cardoso, L, Bradbury, S, Clarke, C, Stears, A, Dover, A, Halsall, D, and R Semple. Diagnosis of insulin autoimmune syndrome using polyethylene glycol precipitation and gel filtration chromatography with ex vivo insulin exchange. Clinical Endocrinology. September 2, 2016; https://www.ncbi.nlm.nih.gov/pubmed/27588366.
  7. Savas-Erdeve, S, Yilmaz Agladioglu, S, Onder A, Peltek Kendirci, HN, Bas VN, Sagsak, E, Cetinkaya, S, and Aycan Z. An uncommon cause of hypoglycemia: insulin autoimmune syndrome. Hormone Research in Paediatrics. 2014; 82(4):278-282. https://www.ncbi.nlm.nih.gov/pubmed/25060673.
  8. Philippon, M, Sejil, S, Mugnier, M, Rocher, L, Guibergia, C, Vialettes, B, and B Delenne. Use of the continuous glucose monitoring system to treat insulin autoimmune syndrome: quantification of glucose excursions and evaluation of treatment efficacy. Diabetic Medicine: A Journal of the British Diabetic Association. July 2014; 31(7):e20-e24. https://www.ncbi.nlm.nih.gov/pubmed/25077287.