INSULIN AUTOIMMUNE SYNDROME (HIRATA DISEASE) - CLINICAL-FEATURES AND EPIDEMIOLOGY IN JAPAN

Since Hirata et al. first reported a patient with insulin autoimmune s yndrome in 1970, 197 cases have been reported in Japan as of December, 1992. The clinical profiles of these 197 cases were as follows; the p eak age at onset was 60-69 years and peak duration of hypoglycemic att acks was more than 1 and less than 3 months. There was no gender diffe rence in the peak age of onset or duration of hypoglycemic attacks. Ap proximately 82% of the IAS patients had spontaneous remission without any positive treatment. Before diagnosis of IAS, 43% of the patients w ith IAS had been taking medication; methimazole (MTZ) for Graves' dise ase, alpha-mercaptopropionyl glycine (MPG) for cataracts, liver diseas e or rheumatoid arthritis, or glutathione for liver disease, all of wh ich are sulfhydryl compounds. After such sulfhydryl compounds were dis continued, the hypoglycemic attacks subsided. Three patients with IAS experienced recurrence of the hypoglycemic attacks after re-administra tion of MTZ and MPG, although 6 patients who developed IAS without exp osure to any drug had recurrent attacks without exposure to any drug a round 1 year after the first hypoglycemic attacks had stopped. Thus, h ypoglycemia in IAS is mainly transient and the development of IAS may be related to sulfhydryl compounds.