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
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In medicine, autoimmune polyendocrine syndromes (APSs), also called autoimmune polyglandular syndromes (APSs), polyglandular autoimmune syndromes (PGASs), or polyendocrine autoimmune syndromes, are a heterogeneous group 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.

Other diseases featuring polycrine autoimmunity:

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.

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