Acta Medica Iranica 2010. 48(3):196-197.

Autoimmune Polyglandular Syndrome Type 2: An Unusual Presentation
Hamdollah Karamifar, Satila Dalili, Zohreh Karamizadeh, Gholamhosein Amirhakimi, Hosein Dalili


Autoimmune polyglandular syndrome (APS) type 2 is characterized by the presence of Addison's disease, in association with autoimmune thyroid disease and/or type 1 diabetes mellitus. APS type 2 occurs most often in middle aged females and is rare in children. Here an 11 year old boy is reported with Addison's disease who developed symptom's of diabetes mellitus, goiter, malabsorption, macrocytic anemia and keratitis. APS type 2 occurs most often in middle aged females and is quite rare in children but one should think to autoimmune poly glandular syndrome type II in patient at any age especially in patients with Addison's disease.


Polyendocrinopathies, autoimmune; Addison disease; diabetes mellitus

Full Text:



Barker JM, Gottlieb PA, Eisenbarth GS. The immuno endocrinopathy syndromes. In: Koronenberg H, editor. Williams Textbook of Endocrinology. Philadelphia: Saunders; 2008. p. 1754-55.

Betterle C, Dal Pra C, Mantero F, Zanchetta R. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocrinol Rev 2002;23(3):327-64.

Zak T, Noczyńska A, Wasikowa R, Zaleska-Dorobisz U, Golenko A. Chronic autoimmune thyroid disease inchildren and adolescents in the years 1999-2004 in Lower Silesia, Poland. Hormones (Athens) 2005;4(1):45-8.

Badenhoop K, Walfish PG, Rau H, Fischer S, Nicolay A, Bogner U, et al. Susceptibility and resistance alleles of human leukocyte antigen (HLA) DQA1 and HLA DQB1 are shared in endocrine autoimmune disease. J Clin Endocrinol Metab 1995;80(7):2112-7.

Papadopoulos KI, Hallengren B. Polyglandular autoimmune syndrome type II in patients with idiopathic Addison's disease. Acta Endocrinol (Copenh) 1990;122(4):472-8.

Betterle C, Volpato M, Greggio AN, Presotto F. Type 2 polyglandular autoimmune disease (Schmidt's syndrome). J Pediatr Endocrinol Metab 1996;9 Suppl 1:113-23.

Papathanasiou A, Kousta E, Skarpa V, Papachileos P, Petrou V, Hadjiathanasiou C. Growth hormone deficiency in a patient with autoimmune polyendocrinopathy type 2. Hormones (Athens) 2007;6(3):247-50.

Perrin C. Disorders of the adrenal gland. In: Behman K, Stanto J, editors. Nelson Textbook of Pediatrics. 18th ed. Philadelphia: Saunders; 2007. p. 357.


  • There are currently no refbacks.

Creative Commons Attribution-NonCommercial 3.0

This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.