Chapter Category: Autoimmunity

From the book Endocrine and Organ Specific Autoimmunity

Insulin Autoimmune Syndrome (IAS, Hirata Disease)

Yasuko Uchigata and Yukimasa Hirata

Although HLA and disease association has been studied for many diseases, only four dis- eases have been identified in which almost all patients have the same HLA antigen; B27 in 88% of ankylosing spondylitis,1 DR4 in 91% of patients with pemphigus vulgaris,2 DR2 in 100% of patients with narcolepsy, DRw52a in 100% of patients with primary sclerosing cholangitis. When the first patient with spontaneous hypoglycemia associated with the production of insulin autoantibodies, so-called insulin autoimmune syndrome (IAS), was reported in Japan by Hirata et al5 in 1970, no one could forecast that IAS was the fifth disease with such a strong HLA-association. Many questions were raised by the first case, including its differential diagnosis from factitious hypoglycemia, the causes of this syndrome, the mechanisms to produce hypoglycemia in this syndrome and so on, raised doubt whether IAS was a “disease“. Immediately after the first patient was diagnosed with IAS, several other patients with the same symptoms and findings were reported over five years. The strong association of IAS with HLA-DR410 gave IAS a citizenship of “disease“, which was named Hirata\'s disease. One hundred and ninety seven Japanese IAS patients have been registered from 1970 to 199211 and a total of 226 Japanese IAS patients have been registered through the end of 1996. Besides the analysis of those reports, several studies concerning the causes of IAS and the hypoglycemia have been clarified by us.

Taken from the book

Endocrine and Organ Specific Autoimmunity

Edited by: Yasuko Uchigata and Yukimasa Hirata

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