Hidekatsu Yanai
National Center for Global Health and Medicine Kohnodai Hospital, Japan
Title: Clinical characteristics of Japanese patients with autoimmune polyglandular syndrome type 3a
Biography
Biography: Hidekatsu Yanai
Abstract
Autoimmune polyglandular syndrome (APS) is an autoimmune disease that involves multiple organ failure. In APS type 3 (APS3), autoimmune thyroid disease occurs with other autoimmune diseases, but not with Addison disease. APS3a is defined as APS3 including autoimmune diabetes. The information about clinical backgrounds of APS3a is very limited. We retrospectively picked up and studied patients with type 1 diabetes and autoimmune diabetes, who showed the positivity for anti-glutamic acid decarboxylase antibody (anti-GAD ab) or anti-islet antigen 2 antibody (anti-IA2 ab) or anti-thyroglobulin antibody (anti-TG ab) or anti-thyroid peroxidase antibody (anti-TPO ab) or anti-thyroid stimulating hormone receptor antibody (anti-TR ab) between January 2010 and January 2016. Present study revealed a remarkable female predominance in APS3a. Among patients with autoimmune diabetes, slowly progressive insulin-dependent diabetes mellitus (SPIDDM) was the most common type of diabetes, and almost 80% of patients with APS3a showed the positivity for anti-GAD ab. Among patients with autoimmune thyroid diseases, almost 80% of patients had possible Hashimoto thyroiditis. Almost 70% of patients with APS3a showed the positivity for anti-TPO ab. Almost 20% of APS3a patients had possible Grave’s disease and 83% of patients with possible Grave’s disease showed overt Grave’s disease. In patients with possible Grave’s disease, the positive rate of anti-TR ab was 100%. In conclusion, we revealed clinical, endocrinological and immunological characteristics of patients with APS3a in Japan.