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Akihiro Nishimura,1 Kimio Matsumura,1 Shota Kikuno,1 Kaoru Nagasawa,1 Minoru Okubo,1 Yasumichi Mori,1 Tetsuro Kobayashi2 1Department of Endocrinology and Metabolism, Toranomon Hospital, Tokyo, Japan; 2Division of Immunology and Molecular Medicine, Okinaka Memorial Institute for Medical Research, Tokyo, JapanCorrespondence: Tetsuro KobayashiOkinaka Memorial Institute for Medical Research, 2-2-2 Toranomon, Minato-Ku, Tokyo, JapanTel +81-3-3588-1111Fax +81-3-3582-7068Email tetsurou@yamanashi.ac.jpAbstract: Slowly progressive type 1 insulin-dependent diabetes mellitus (SPIDDM), sometimes referred to as latent autoimmune diabetes in adults (LADA), is a heterogeneous disease that is often confused with type 1 and type 2 diabetes. As a result, there were few diagnostic criteria for this disorder until 2012, when the Japan Diabetes Society established criteria that could be used in clinical practice. A primary question is whether pathologic markers for type 1 or type 2 diabetes are present in the pancreas of patients with SPIDDM, because the phenotype of SPIDDM is similar to both type 1 and type 2 diabetes. Recent studies clarified pathologic findings in the pancreas of patients with SPIDDM, which included T-cell-mediated insulitis, a marker of type 1 diabetes; pseudoatrophic islets (islets specifically devoid of beta cells), another hallmark of type 1 diabetes; and a lack of amylin (ie, islet amyloid polypeptide) deposition to the islet cells, a pathologic marker of type 2 diabetes. In terms of preventing the loss of beta-cell function in patients with SPIDDM, several studies have shown that some drugs, including dipeptidyl peptidase-4 inhibitors, are effective. There is an increased need for early diagnosis of SPIDDM to preserve beta-cell function. This review presents updated findings on the pathogenesis and immunologic findings of the affected pancreas, diagnostic markers, risk factors for progression of beta-cell dysfunction, epidemiology, clinical features, diagnostic strategies, prevention strategies, and clinical options for patients with SPIDDM.Keywords: slowly progressive type 1 diabetes mellitus; SPIDDM, latent autoimmune diabetes in adults; LADA, insulitis, PanIN, GAD antibody |
