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   - March 14, 2010
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 Presentation

"Polyglandular Autoimmune Syndromes"

Dr. Patricia Crock (biography)
English - 2008-05-22 - 49 minutes
(46 slides)

Summary :
Dr. Crock starts with a case presentation of a boy with Addison's disease, glucocorticoid and mineralocorticoid deficiency, and a family history of polyglandular autoimmune disease.

Autoimmune endocrinopathies cluster, for example patients with type 1 diabetes may have Hashimoto's disease or coeliac disease. Although Addison's disease is rare it may be indicated by falling insulin doses. Lymphocytic hypophysitis, although very rare in children should be considered in women with type 1 diabetes in pregnancy. There are some non-organ specific autoimmune diseases that also cluster with diabetes, such as systemic lupus erythematosus, rheumatoid arthritis, and inflammatory bowel disease.

Autoimmune Polyendocrine Syndromes (APS), were classified in 1981 by Neufeld and Blizzard. APS type 1 is also known as APECED (Autoimmune PolyEndocrinopathy – Candidiasis – Ectodermal Dystrophy). APS type 2 is autoimmune Addison's disease with autoimmune thyroid disease and/or type 1 diabetes mellitus. APS type 3 is autoimmune thyroid disease with other organ specific autoimmunity (excluding Addison's disease). The rare monogenic forms of autoimmunity IPEX ( Immune dysfunction Polyendocrinopathy Enteropathy X-linked) and ALPS (Autoimmune Lymphoproliferative Syndromes) are also discussed. Dr. Crock talks about underlying immunological concepts such as the role of the thymus and central versus peripheral tolerance, and describes the syndromes in detail.

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Learning objectives :
After viewing this presentation the participant will be able to discuss:
- Autoimmune diseases which cluster with type 1 diabetes
- A classical case presentation of polyglandular autoimmune disease
- Target autoantigens and autoantibody markers
- How to avoid some diagnostic pitfalls
- Monogenic Autoimmunity Syndromes
- Polyglandular Autoimmune syndromes

   


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