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Year : 2014  |  Volume : 18  |  Issue : 3  |  Page : 430-431

A very rare cohort of elderly patients with autoimmune polyglandular syndrome type 3b

Department of Internal Medicine and Geriatrics, Center Hospital University of Reims, Reims, France

Date of Web Publication3-May-2014

Correspondence Address:
Zulfiqar Abrar-Ahmad
45 Rue Cognacq-Jay, Reims - 51100
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2230-8210.131226

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How to cite this article:
Abrar-Ahmad Z. A very rare cohort of elderly patients with autoimmune polyglandular syndrome type 3b. Indian J Endocr Metab 2014;18:430-1

How to cite this URL:
Abrar-Ahmad Z. A very rare cohort of elderly patients with autoimmune polyglandular syndrome type 3b. Indian J Endocr Metab [serial online] 2014 [cited 2021 Jun 13];18:430-1. Available from: https://www.ijem.in/text.asp?2014/18/3/430/131226


Autoimmune polyglandular syndrome was described by Neufeld et al. as an autoimmune disease that involves multiorgan failure. [1],[2] They comprise a wide spectrum of autoimmune diseases, [3] and encompass a rare juvenile type polyglandular autoimmune syndrome type 1 and a more frequent adult types 2 and 3. [4] Polyglandular autoimmune syndrome type 2 is defined as the association between Addison's disease and either autoimmune thyroid disease or type 1 diabetes and other autoimmune diseases like pernicious anemia; polyglandular autoimmune syndrome type 3 is characterized by the main syndrome which is autoimmune thyroiditis. It can either join an autoimmune diabetes (and more or less sarcoidosis or celiac disease) defining the type 3a; either pernicious anemia defining the type 3b; either vitiligo and alopecia defining 3c. [5] It differs from the type 2 by the absence of adrenal insufficiency. The incidence of polyglandular autoimmune syndrome type 2 and 3 peak at ages 20 to 60 years, mostly in the third of fourth decade. [4] We describe here a very rare cohort of patients aged 65 years and more, with autoimmune polyendocrinopathy 3b.

We performed a two-center study in Reims and Strasbourg University Hospital, retrospective, collecting cases of pernicious anemia diagnosed after 65 years, associated with autoimmune diseases in the elderly. Twenty-eight patients are diagnosed pernicious anemia associated with other autoimmune diseases. The mean age is 76.6 years; the female-to-male ratio is 3:1. We noted two cases where pernicious anemia is associated with adrenal insufficiency, autoimmune thyroiditis and autoimmune diabetes (inconsistently), defining the type 2. What emerges from our study is that pernicious anemia fits more frequently in the type 3b, with 19 cases of association pernicious anemia and autoimmune thyroiditis.

Polyglandular autoimmune syndrome type 3 is characterized by a complex inheritance pattern. At least three genes are involved as major susceptibility genes: HLA class II, CTLA-4, and PTPN22. [4] Pernicious anemia has an association with HLA-DR5 for a relative risk of 5, Hashimoto's thyroiditis partnering with the HLA-DR3 to a relative risk of 3.2 and HLA-DR5 for a relative risk 58, [4] the autoimmune diabetic subjects, 90% of the Caucasian population associated with HLA DR3 and/or DR4. [4] Many studies indicate that both HLA haplotypes DR3-DQB1*0201 and DR4-DQ*0302 contribute to the polyglandular autoimmune syndrome type 3 (4). A genetic predisposition has been suggested for pernicious anemia. In type 1 diabetic subjects, a weak association between pernicious anemia and HLA haplotype DQA1 *0501-B1 *0301, HLA-DR5 bound was observed. [4],[5] Patients with pernicious anemia association and autoimmune endocrine diseases often have DR3/DR4 genotype. Autoimmune thyroiditis/pernicious anemia is part of a typical polyendocrinopathy 3b for which a predisposition genetic (HLA-B8 and/or DR3 and DR5) exist.

We can conclude that early detection of antibodies and latent organ-specific dysfunction is advocated to alert physicians to take appropriate action in order to prevent full-blown disease in the elderly. Family members of elderly patients should be regularly screened.

   References Top

1.Neufeld M, Maclaren N, Blizzard R. Autoimmune polyglandular syndromes. Pediatr Ann 1980;9:154-62.  Back to cited text no. 1
2.Anderson MS. Update in endocrine autoimmunity. J Clin Endocrinol Metab 2008;93:3663-70.  Back to cited text no. 2
3.Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med 2004;350:2068-79.  Back to cited text no. 3
4.Kahaly GJ. Polyglandular autoimmune syndrome type II. Presse Med 2012;41:e663-70.  Back to cited text no. 4
5.Humbel RL. Polyglandular autoimmune syndrome. Revue De L′ Acomen 1999;5:271-5.  Back to cited text no. 5

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