Autoimmune polyendocrinopathy

From Citizendium
Jump to navigation Jump to search
The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.
This article is a stub and thus not approved.
Main Article
Discussion
Related Articles  [?]
Bibliography  [?]
External Links  [?]
Citable Version  [?]
 
This editable Main Article is under development and subject to a disclaimer.

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 (2024), 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.