Clinical, Endocrinological and Immunological Characteristics of Japanese Patients With Autoimmune Polyglandular Syndrome Type 3a

Sumie Moriyama, Reo Yoshikawa, Hisayuki Katsuyama, Hidetaka Hamasaki, Hiroki Adachi, Hidekatsu Yanai


Background: Autoimmune polyglandular syndrome (APS) is an autoimmune disease that involves multiple organ failure. In 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 studied to understand clinical, endocrinological and immunological characteristics of Japanese patients with APS3a.

Methods: We reviewed our previously published case reports about APS3a, and picked up 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. We collected data including age, sex, anti-GAD ab, anti-IA2 ab, HbA1c, serum fasting C-peptide immunoreactivity (CPR), urinary CPR, treatment for diabetes, anti-TPO ab, anti-TG ab, anti-TR ab, thyroid stimulating hormone (TSH), free triiodothyronine (fT3), free thyroxine (fT4), findings of thyroid ultrasonography and treatment for thyroid diseases.

Results: 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 Graves disease, and 83% of patients with possible Graves disease showed overt Graves disease. In patients with possible Graves disease, the positive rate of anti-TR ab was 100%.

Conclusion: We revealed clinical, endocrinological and immunological characteristics of patients with APS3a in Japan.

J Endocrinol Metab. 2016;6(2):46-51


Autoimmune polyglandular syndrome 3a; Insulin; Thyroid diseases; Type 1 diabetes

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