Background and Methods Type 1 diabetes mellitus is now classifiedas autoimmune (type 1A) or idiopathic (type 1B), but littleis known about the latter. We classified 56 consecutive Japaneseadults with type 1 diabetes according to the presence or absenceof glutamic acid decarboxylase antibodies (their presence isa marker of autoimmunity) and compared their clinical, serologic,and pathological characteristics.
Results We divided the patients into three groups: 36 patientswith positive tests for serum glutamic acid decarboxylase antibodies,9 with negative tests for serum glutamic acid decarboxylaseantibodies and glycosylated hemoglobin values higher than 11.5percent, and 11 with negative tests for serum glutamic aciddecarboxylase antibodies and glycosylated hemoglobin valueslower than 8.5 percent. In comparison with the first two groups,the third group had a shorter mean duration of symptoms of hyperglycemia(4.0 days), a higher mean plasma glucose concentration (773mg per deciliter [43 mmol per liter]) in spite of lower glycosylatedhemoglobin values, diminished urinary excretion of C peptide,a more severe metabolic disorder (with ketoacidosis), higherserum pancreatic enzyme concentrations, and an absence of islet-cell,IA-2, and insulin antibodies. Immunohistologic studies of pancreatic-biopsyspecimens from three patients with negative tests for glutamicacid decarboxylase antibodies and low glycosylated hemoglobinvalues revealed T-lymphocytepredominant infiltrates inthe exocrine pancreas but no insulitis and no evidence of acuteor chronic pancreatitis.
Conclusions Some patients with idiopathic type 1 diabetes havea nonautoimmune, fulminant disorder characterized by the absenceof insulitis and of diabetes-related antibodies, a remarkablyabrupt onset, and high serum pancreatic enzyme concentrations.
Source Information
From the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, Osaka University, Osaka, Japan.
Address reprint requests to Dr. Imagawa at the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, B5, Osaka University, 2-2 Yamadaoka, Suita 565-0871, Japan, or at imagawa{at}imed2.med.osaka-u.ac.jp.
A Novel Subtype of Type 1 Diabetes Mellitus
Honeyman M. C., Coulson B. S., Harrison L. C., Tanaka S., Kobayashi T., Momotsu T., Imagawa A., Miyagawa J.-i., Hanafusa T.
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N Engl J Med 2000;
342:1835-1837, Jun 15, 2000.
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