Fulminant Type 1 Diabetes

TLDR

The study aimed to characterize the clinical and immunologic features of fulminant type 1 diabetes through a nationwide survey. Researchers examined history, laboratory data, and islet‑related autoantibodies in 222 hospitalized patients and an additional 118 out‑of‑hospital patients, totaling 161 fulminant cases across Japan. Among 222 hospitalized patients, 19.4% had fulminant type 1 diabetes, and an additional 118 cases from outside hospitals brought the total to 161, with fulminant patients showing higher rates of flu‑like symptoms, pregnancy, and more severe metabolic derangement, 4.8% anti‑GAD positivity, and accounting for roughly 20% of ketosis‑onset type 1 diabetes in Japan.

Abstract

OBJECTIVE— To describe the clinical and immunologic characteristics of fulminant type 1 diabetes, a novel subtype of type 1 diabetes, we conducted a nationwide survey. RESEARCH DESIGN AND METHODS—History and laboratory data, including islet-related autoantibodies, were examined in 222 patients with fulminant and nonfulminant type 1 diabetes in our hospitals in addition to another 118 patients with fulminant type 1 diabetes located outside our hospitals in Japan. RESULTS—In our hospitals, of the 222 patients studied, 43 (19.4%) were diagnosed with fulminant type 1 diabetes, 137 (61.7%) were classified as having autoimmune type 1 diabetes, and 42 were type 1 diabetic subjects who were not fulminant and did not have anti-islet antibodies. An additional 118 fulminant patients outside our hospitals were enrolled, making a total of 161 fulminant type 1 diabetic subjects (83 male and 78 female subjects; 14 children/adolescents and 147 adults) identified from all over Japan. (In 2000, the average incidence was three cases per month.) Flu-like symptoms and pregnancy were more frequently observed in the fulminant than in the autoimmune group (P < 0.001). In the fulminant patients, 4.8% were positive for anti-GAD antibodies and none were positive for anti–islet antigen 2 antibodies. CONCLUSIONS—Fulminant type 1 diabetes is a distinct subtype and accounts for ∼20% of the ketosis-onset type 1 diabetes cases in Japan. Flu-like symptoms are characteristic of disease onset. Metabolic derangement is more severe in this subtype than in autoimmune type 1 diabetes.