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Polyglandular autoimmune syndrome I

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

This is an autosomal recessive polyglandular deficiency state characterised by childhood onset and defective cellular immunity to Candida albicans which is refractory to conventional chemotherapy; however remission is reported with ketoconazole plus transfer factor. There is no HLA linkage.

The autoimmune element comprises:

  • hypoparathyroidism is common
  • diabetes is rare
  • adrenal insufficiency may develop acutely
  • pernicious anaemia
  • chronic active hepatitis
  • membranoproliferative glomerulonephritis
  • sprue

The associated disorders must be managed conventionally even if the Candida is successfully treated.

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