Purpose : We clarified epidemiological and clinical characteristics and set diagnostic criteria for fulminant type 1 diabetes, a novel subtype of type 1 diabetes mellitus. The Japan Diabetes Society Committee on Fulminant Type 1 Diabetes Mellitus Research was organized for this study.
Methods : The committee conducted 2 studies—Study 1 within institutions related to committee members and to reveal the prevalence of fulminant type 1 diabetes in Japan, and Study 2 conducted nationwide to clarify clinical characteristics of fulminant type 1 diabetes. The committee asked members of the Japan Diabetes Society to report patients suspected of fulminant type 1 diabetes mellitus. Characteristics of these patients were compared to classical autoimmune type 1 diabetes mellitus. Following these 2 studies, the committee drew up screening and diagnostic criteria for fulminant type 1 diabetes mellitus.
Results : In Study 1, the committee identified 222 patients with acute-onset type 1 diabetes accompanied with ketoacidosis. Of these, 43 (19.4%) were diagnosed with fulminant type 1 diabetes mellitus. In Study 2, the committee diagnosed an additional 118 patients with fulminant type 1 diabetes mellitus outside institutions of committee members. In total, 161 were diagnosed with fulminant type 1 diabetes mellitus, which showed no significant seasonal or regional preference. Compared to 137 autoimmune type 1 diabetes mellitus, fulminant type 1 diabetes mellitus had a significantly shorter duration of hyperglycemic symptoms, a higher prevalence of preceding symptoms, e.g., flu-like symptoms (fever, sore throat etc.) and gastrointestinal symptoms (epigastralgia, nausea, vomiting, etc.), a higher prevalence of disturbance in consciousness, higher plasma glucose, lower HbA₁c, more frequent occurrence related to pregnancy, higher prevalence of increased serum pancreatic enzyme, lower C-peptide in serum and urine, lower arterial pH, lower serum Na, and higher serum K. Autoantibodies against constituents of pancreatic beta-cells, hallmarks of autoimmune type 1 diabetes mellitus, were negative in 95.2% of patients with fulminant type 1 diabetes mellitus. The other 4.8% were positive for GAD antibody, but in general the titer was low. Based on these 2 studies, the committee set up both “screening criteria” for catching the disease and “diagnostic criteria” for definitely diagnosing it.
Conclusion : This study clarified epidemiological and clinical characteristics of fulminant type 1 diabetes mellitus, which differ from autoimmune type 1 diabetes. The committee set up screening and diagnostic criteria. Fulminant type 1 diabetes mellitus, if it is disregarded or not caught, results directly in death of the patient. We believe that these criteria will help saving lives of patients with this rapidly-progressing type of diabetes.

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We studied 4 cases of diagnosed or suspected fulminant type 1 diabetes mellitus based on clinical factors, i.e., age, gender, pregnancy, duration between onset of symptoms and diagnosis, HbA₁c, autoimmune antibodies, and HLA. Of the 4, 3 were women and a man, and one woman was pregnant. Age ranged from 29 to 69. Cases 3 and 4 had upper bronchitis before hyperglycemic symptom onset. The duration was 2 to 5 days, and the shorter the duration, the lower the HbA₁c. None had antibodies against glutamic acid decarboxylase, ICA, or IAA, and only case 1 had ICSA. Three of the 4 had HLA DRB1^*0405-DQB1^*0401, as haplotype susceptible to type 1 diabetes in Japanese. Taken together, we suggest that fulminant type 1 diabetes mellitus may be related to a variety of factors, e.g., gender, pregnancy, virus infection and autoimmunity. We also emphasize the importance of the HLA DRB1^*0405-DQB1^*0401 haplotype in fulminant type 1 diabetes mellitus onset.

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