Autoimmune polyendocrinopathy

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In endocrinology, autoimmune polyendocrinopathies, also called polyglandular autoimmune syndrome or autoimmune polyendocrine syndrome (APS), are "autoimmune diseases affecting multiple endocrine organs.[1][2]

Classification

  • "Type I is characterized by childhood onset and chronic mucocutaneous candidiasis (candidiasis, chronic mucocutaneous)."[1]
  • "Type II exhibits any combination of adrenal insufficiency (addison's disease), lymphocytic thyroiditis (thyroiditis, autoimmune;), hypoparathyroidism; and gonadal failure."[1] Type II is also called Schmidt's Syndrome if Addison disease is present.
  • IPEX[3]

Etiology/cause

"In both types organ-specific antibodies against a variety of endocrine glands have been detected. The type II syndrome differs from type I in that it is associated with HLA-A1 and B8 haplotypes, onset is usually in adulthood, and candidiasis is not present."[1]

Notable cases

American President John F. Kennedy may have had this syndrome.[4]

References

  1. 1.0 1.1 1.2 1.3 Anonymous (2015), Autoimmune polyendocrinopathy (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes.". N Engl J Med 350 (20): 2068-79. DOI:10.1056/NEJMra030158. PMID 15141045. Research Blogging.
  3. d'Hennezel E, Ben-Shoshan M, Ochs HD, Torgerson TR, Russell LJ, Lejtenyi C et al. (2009). "FOXP3 forkhead domain mutation and regulatory T cells in the IPEX syndrome.". N Engl J Med 361 (17): 1710-3. DOI:10.1056/NEJMc0907093. PMID 19846862. Research Blogging.
  4. Mandel LR (2009). "Endocrine and autoimmune aspects of the health history of John F. Kennedy.". Ann Intern Med 151 (5): 350-4. PMID 19721023.