Early recognition of polyglandular autoimmune syndrome type II

Polyglandular autoimmune syndromes (PGA) are uncommon constellations of organ-specific autoimmune diseases, characterized by the existence of more than one autoimmune disease in an affected individual. PGA type II (Schmidt’s Syndrome) is characterized by the association of autoimmune Addison’s disease with thyroid autoimmune diseases and/or type 1 diabetes mellitus. Here, we report a 12.5 years old girl who applied to our clinic with the complaint of hyperpigmentation of the skin, malaise for the last 5 months and also suffering from dizziness and excessive sweating. Adrenal insufficiency was thought to be the diagnosis. Further laboratory tests revealed an increased basal ACTH level, and a blunted cortisol response to ACTH stimulation. Autoantibodies against thyroid and adrenal gland were elevated. Therefore, she was diagnosed as PGA type II autoimmune thyroiditis and autoimmune Addison’s disease. Polyglandular autoimmune syndrome type II which usually being in older age groups, is very rare at this age. Early recognition of these components of the syndrome and replacement therapy can be life saving, particularly when there is adrenal or thyroid insufficiency.

___

1. Neufeld M, Mac Laren N, Blizzard R. Autoimmune polyglandular syndromes. Pediatr Ann 1980;9:154-63.

2. Hügle B, Döllman R, Keller E, Kiess W. Addison’s crisis in adolescent patients with previously diagnosed diabetes mellitus as manifestation of polyglandular autoimmune syndrome type II-Report of two patients. J Pediatr Endocrinol Metab 2004;17: 93-7.

3. Muir A, She JX. Advances in the genetics and immunology of autoimmune polyglandular syndrome II/III and their clinical applications. Ann Med Interne 1999; 150:301-12.

4. Betterle C, Dal Pra C, Montero F, Zanchetta R. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocrinol Rew 2002 ; 23: 237-364.

5. Falorni A, Lauretti S. Adrenal autoimmunity and correlation with adrenal dysfunction. The Endocrinologist 2000; 10:145-54.
Yeni Tıp Dergisi-Cover
  • ISSN: 1300-2317
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 2018
  • Yayıncı: -
Sayıdaki Diğer Makaleler

A 54-year-old male patient with amaurosis fugax

Haksun EBİNÇ, Mesut SEZİKLİ, Ayerden Fatma EBİNÇ

Neonatal resüsitasyon konusunda sağlık çalışanlarının düşünceleri ve pratik uygulamaları

Didem ALİEFENDİOĞLU, Cihat ŞANLI, Dilek Emine MISIRLIOĞLU, MERYEM ALBAYRAK, Şule TOPRAK, Hızel Selda BÜLBÜL

Anorektal malformasyon tespit edilen 51 yaşında kadın hasta

Erkan CÜRE, Arzu DENLER, Muhammed Cem KOÇKAR, Altuğ ŞENOL, Abdulkadir BAŞTÜRK, Bünyamin AYDIN

Urinary N-acetyl- β -D glucosaminidase activity in newborns as a marker of hypoxic-ischemic tubular damage

Kibriya FİDAN, Nilgün ÇAKAR, Didem ALİEFENDİOĞLU, Hulusi KOÇAK, Fatma Nur ŞENİZ

Epidermodisplazya verrusiformis: İki olgu

Muhterem POLAT, Emine TAMER, Şafak KÖLÜKFAKI, Güneş GÜR, Hüseyin ÜSTÜN, Nuran ALLI

Remifentanil nörolept anestezide fentanile alternatif olabilir mi ?

Bünyamin MUSLU, Selda MUSLU, R.Dilek ZENGİN, Ayşegül ÖZKÖK, Özcan ERDEMLİ

Travma hastalarında ultrasonografi ve bilgisayarlı tomografi sonuçlarının karşılaştırılması

AYDIN ÜNAL, Pınar YAZICI, Alper UĞUZ, Sadık TAMSEL, İlhami SOLAK, Hasan KAPLAN

Sürekli ayaktan periton diyaliz programındaki bir hastada tüberküloz peritoniti

Burak UZ, Mehmet KANBAY, Ali AKÇAY

Çocukluk çağında allerjik rinit

Ferhat ÇATAL, Emin METE, Cüneyt TAYMAN, Semra KARA

Early recognition of polyglandular autoimmune syndrome type II

Ergun ÇETİNKAYA, Zehra AYCAN, Ebru GÖZER, Ebru PETEK, Ayşe Esin KİBAR