Autoimmune polyendocrine syndrome

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Autoimmune polyendocrine syndrome
Classification and external resources
ICD-10E31.0
ICD-9258.1
OMIM240300 269200
DiseasesDB29212 29690
eMedicinemed/1867 med/1868
MeSHD016884
 
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Autoimmune polyendocrine syndrome
Classification and external resources
ICD-10E31.0
ICD-9258.1
OMIM240300 269200
DiseasesDB29212 29690
eMedicinemed/1867 med/1868
MeSHD016884

In medicine, autoimmune polyendocrine syndromes, also called polyglandular autoimmune syndrome (PGAS),[1] are a heterogeneous group[2] of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected.

There are three "autoimmune polyendocrine syndromes", and a number of other diseases which have endocrine autoimmunity as one of their features.

The syndromes[edit]

Other diseases[edit]

Other diseases featuring polycrine autoimmunity:

Management[edit]

In principle, the component diseases are managed as usual. The challenge is to detect the possibility of any of the above syndromes, and to anticipate other manifestations. For example, in a patient with known Type 2 autoimmune polyendocrine syndrome but no features of Addison's disease, regular screening for antibodies against 21-hydroxylase (a feature of Addison's) may prompt early intervention and hydrocortisone replacement to prevent characteristic crises.

References[edit]

  1. ^ "Polyglandular Autoimmune Syndromes: Immunogenetics and Long-Term Follow-Up". Retrieved 1 July 2013. 
  2. ^ Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes". N. Engl. J. Med. 350 (20): 2068–79. doi:10.1056/NEJMra030158. PMID 15141045. 
  3. ^ Yong PL, Russo P, Sullivan KE (May 2008). "Use of Sirolimus in IPEX and IPEX-Like Children". J. Clin. Immunol. 28 (5): 581–7. doi:10.1007/s10875-008-9196-1. PMID 18481161.