Autoimmune polyendocrine syndrome

Autoimmune polyendocrine syndrome
Classification and external resources
Specialty endocrinology
ICD-10 E31.0
ICD-9-CM 258.1
OMIM 240300 269200
DiseasesDB 29212 29690
eMedicine med/1867 med/1868
MeSH D016884

In medicine, autoimmune polyendocrine syndromes (APSs), also called autoimmune polyglandular syndromes (APSs), polyglandular autoimmune syndromes (PGASs),[1] or polyendocrine autoimmune syndromes, 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 types of APS or (in terms that mean the same thing) three APSs, and there are a number of other diseases which have endocrine autoimmunity as one of their features.

The syndromes

Other diseases

Other diseases featuring polycrine autoimmunity:

Management

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

  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.
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