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Type III polyglandular autoimmune syndrome
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Type III polyglandular autoimmune syndrome

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Synopsis

A rare polygenetic condition consisting of an endocrinopathy, excluding Addison disease, in combination with an organ-specific autoimmune thyroid disorder (either Hashimoto thyroiditis or Graves disease). It is a result of poor hormone production due to defective endocrine glands. Etiology is not well understood, but genetic inheritance is possible. Occurs mostly in middle-aged women. There are 3 variations based on the specific comorbid endocrinopathy: autoimmune thyroiditis paired with 1) type 1 diabetes mellitus; 2) pernicious anemia; or 3) vitiligo, alopecia, or another organ-specific disorder such as celiac disease, hypogonadism, or myasthenia gravis.

Codes

ICD10CM:
E31.0 – Autoimmune polyglandular failure

SNOMEDCT:
449731009 – Autoimmune polyendocrine syndrome type 3

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Last Updated: 08/26/2016
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Type III polyglandular autoimmune syndrome
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Type III polyglandular autoimmune syndrome (PAS III/T1DM with Hashimoto Thyroiditis)
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