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polyglandular autoimmune syndrome I
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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.
Last reviewed 01/2018
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