Rare Pulmonology News

Disease Profile

Insulin autoimmune syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

ageofonset-adult.svg

ICD-10

E16.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

notapplicable.svg

Other names (AKA)

Hirata disease; Insulin autoimmune hypoglycemia

Summary

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]

Symptoms

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]

Cause

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] 

Diagnosis

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] 

Treatment

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] 

Organizations

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

    Learn more

    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.

      References

      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. https://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. https://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.

      Rare Pulmonology News