Polyglandular autoimmune syndromes (PGA) are rare immune endocrinopathies characterised by the coexistence of at least 2 endocrine gland insufficiencies/poorly functioning that are based on autoimmune mechanisms.
Associations with nonendocrine immune diseases also occur.
PGA can be classified as:
Type I (PGA-I) (polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), Whitaker syndrome)
Rare autosomal-recessive disorder that first manifests in early childhood and results in tissue-specific multiorgan autoimmunity, leading to decreased functioning of multiple glands.
Endocrine organs such as the adrenal cortex, ovaries, and parathyroid glands are typically affected, which results in various clinical presentations, including:
Disorders of the parathyroid hormone gland and related hormones (hypoparathyroidism- where not enough parathyroid hormone gland is produced or Pseudohypoparathyroidism when there is resistance shown by the end -organs to the parathyroid hormone)
Neck irradiation/radioiodine therapy
Postparathyroidectomy in dialysis patients
Inadvertent surgical removal (can be transient or permanent)
Disorders of the parathyroid hormone gland and related hormones (hypoparathyroidism- where not enough parathyroid hormone gland is produced or Pseudohypoparathyroidism when there is resistance shown by the end -organs to the parathyroid hormone)
Neck irradiation/radioiodine therapy
Postparathyroidectomy in dialysis patients
Inadvertent surgical removal (can be transient or permanent)
Type I (PGA-I)(polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), Whitaker syndrome)
Mutations in the autoimmune regulator gene, located on chromosome 21q22.3
Family history-autosomal recessive disorder (Both parents who are carriers of the recessive genes responsible and can cause a 25% chance of their sibling getting this condition)
If both parents are carriers, a child has a 25 percent chance of inheriting two recessive genes (and getting the condition), a 50 percent chance of getting one dominant and one recessive gene (and becoming a carrier), and a 25 percent chance of getting two dominant genes (and remaining unaffected).
Family history-autosomal recessive disorder (Both parents who are carriers of the recessive genes responsible and can cause a 25% chance of their sibling getting this condition)
If both parents are carriers, a child has a 25 percent chance of inheriting two recessive genes (and getting the condition), a 50 percent chance of getting one dominant and one recessive gene (and becoming a carrier), and a 25 percent chance of getting two dominant genes (and remaining unaffected).
Source: https://www.nejm.org 'symptoms depend on type of syndrome'
Source: ResearchGate
Complications /Information to beware of/General tips:
Please copy and paste any key words from the title: Polyglandular autoimmune syndromes (PGA) (APECED, Whitaker syndrome,Schmidt syndrome) in the following respective 'Medtick References and/or Sources' to find out more about the disease (this also may include diagnosis tests and generic medical treatments).
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