{"id":324128,"date":"2022-11-22T14:00:00","date_gmt":"2022-11-22T13:00:00","guid":{"rendered":"https:\/\/medizinonline.com\/are-there-signs-of-autoimmune-polyendocrine-syndrome\/"},"modified":"2022-11-22T14:00:00","modified_gmt":"2022-11-22T13:00:00","slug":"are-there-signs-of-autoimmune-polyendocrine-syndrome","status":"publish","type":"post","link":"https:\/\/medizinonline.com\/en\/are-there-signs-of-autoimmune-polyendocrine-syndrome\/","title":{"rendered":"Are there signs of autoimmune polyendocrine syndrome?"},"content":{"rendered":"

Patients with type 1 diabetes have a higher risk of developing other autoimmune diseases. The autoimmune-related polyendocrine syndromes are rare but should be detected in time. Certain genetic mutations are predisposing. Antibody determination plays an important role in diagnosis and therapy.<\/strong><\/p>\n

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In autoimmune polyendocrine syndromes (APS), autoimmune reactions take place against various endocrine organs, ultimately leading to their failure. APS type 2 usually occurs in young adulthood<\/strong>, but there are exceptions with earlier onset (box)<\/span>. Women are estimated to be three times more frequently affected [1]. The main components of APS type 2 include Addison’s disease, autoimmune thyreopathy, and diabetes mellitus type 1 [2]. Other associated autoimmune diseases include celiac disease, myasthenia gravis, vitiligo, alopecia, and pernicious anemia [2]. The co-occurrence of type 1 diabetes and Addison’s disease is particularly dangerous clinically because it can complicate the counter-regulation of hypoglycemia [7]. APS runs in families. A multifactorial etiology and a polygenetic predisposition are assumed. Several years may elapse between the manifestation of each glandular autoimmune disease and the full-blown APS. Serologic screening of patients at risk for autoimmune disease has been proposed to detect and treat APS early [8]. Autoimmune and genetic testing can be helpful for this purpose. APS type 2 is inherited in an autosomal dominant manner with incomplete penetrance and correlates with different HLA alleles. Genes in the HLA complex on chromosome 6 have been identified as the most important risk factor, particularly HLA-DRB1*03, HLA-DRB1*04, and DQB1*02 [9]. Regarding detection of autoantibodies in serum by indirect immunofluorescence and specific immunoassays, 21-hydroxylase (21-OH) for Addison’s disease, as well as various diabetes- and thyroid-specific autoantibodies are of particular importance (Table 1)<\/span> [2,5]. In the treatment of APS type 2, the focus is on guideline-based substitution of the missing hormones of the respective existing diseases (Addison’s disease, autoimmune thyreopathy, diabetes mellitus type 1) [3].<\/p>\n

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In 95% of cases, autoantibodies against pancreatic islet cells, insulin, glutamate decarboxylase (GAD), and tyrosine phosphatase are detectable in the initial manifestation of type 1 diabetes (T1D) [6]. These autoantibodies can be detected months or years before clinical symptoms of diabetes [6]. In a study by Savvateeva et al. antibody profiles of patients with autoimmune polyendocrine syndromes (APS) with and without diabetes were analyzed and compared to a healthy control group. For this purpose, blood serum samples from 206 subjects aged 18-88 years were studied: patients with APS type 1 (n=18), APS type 2 (n=39), isolated autoimmune endocrine pathology (n=50) or non-autoimmune endocrine pathology (n=71), healthy controls (n=28). The microarray-based assays revealed differences with respect to the prevalence of autoantibodies. A significantly higher proportion of patients with APS type 2 with or without T1D had at least one of the three autoantibodies GAD-65, IA-2, or ICA compared to the healthy control group (Fig. 1)<\/span>.<\/strong> A similar pattern was seen with respect to the two autoantibodies TG or TPO, comparing here APS patients with vs. without AITD with a healthy control group. Thus, at least one of the two autoantibodies TG or TPO was more frequently detected in blood serum samples of patients with APS type 2 with and without AITD [5]. The authors highlight that none of the patients with either sole or APS-2-associated diabetes tested positive for all three diabetes-related antibodies (GAD, ICA, IA-2).<\/p>\n

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Literature:<\/p>\n

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  1. Grossmann BM: Prevalence of rheumatologic-immunologic diseases in the setting of premature ovarian failure (POF). Inaugural Dissertation, 2018, https:\/\/d-nb.info\/1227973527\/34, (last accessed Sept. 28, 2022).<\/li>\n
  2. Herold M, Conrad K, Sack U (eds.): Autoimmune Diseases – A Guide for Family Physicians, Translated and edited edition of The General Practice Guide to Autoimmune Diseases, edited by Y. Shoenfeld and P. L. Meroni, Pabst Science Publishers 2012.<\/li>\n
  3. Sperling MA, Angelousi A, Yau M: Autoimmune polyglandular syndromes. [Updated 2021 Apr 10]. In: Feingold KR, Anawalt B, Boyce A, et al, eds. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc; 2000. www.ncbi.nlm.nih.gov\/books\/NBK279152, (last accessed Sept 28, 2022).<\/li>\n
  4. Hansen MP, Erlich M, Kahaly GJ: Addison’s disease and pluriglandular syndromes. Journal of Clinical Endocrinology and Metabolism 2013; 6(2), 5-11.<\/li>\n
  5. Savvateeva EN, et al: Multiplex Autoantibody Detection in Patients with Autoimmune Polyglandular Syndromes. Int J Mol Sci 2021; 22(11): 5502.<\/li>\n
  6. Hansen MP, Matheis N, Kahaly GJ: Type 1 diabetes and polyglandular autoimmune syndrome: A review. World J Diabetes 2015 Feb 15; 6(1): 67-795.<\/li>\n
  7. Lehnert H: Rational diagnostics and therapy in endocrinology, diabetology and metabolism. 2015, www.thieme-connect.de\/products\/ebooks\/lookinside\/10.1055\/b-0035-104807, (last accessed Sept. 28, 2022)<\/li>\n
  8. Kuschnereit M: Demography and clinic of polyglandular autoimmune syndrome, PhD thesis, 2020, https:\/\/openscience.ub.uni-mainz.de\/, (last accessed Sept 28, 2022).<\/li>\n
  9. Flesch BK, et al: HLA class II haplotypes differentiate between the adult autoimmune polyglandular syndrome types II and III. The Journal of clinical endocrinology and metabolism 2014; 99(1): E177-182.<\/li>\n<\/ol>\n

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    HAUSARZT PRAXIS 2022; 17(10): 42-44<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"

    Patients with type 1 diabetes have a higher risk of developing other autoimmune diseases. The autoimmune-related polyendocrine syndromes are rare but should be detected in time. Certain genetic mutations are…<\/p>\n","protected":false},"author":7,"featured_media":126542,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"pmpro_default_level":"","cat_1_feature_home_top":false,"cat_2_editor_pick":false,"csco_eyebrow_text":"Diabetes mellitus type 1 ","footnotes":""},"category":[11508,11384,11297,11323,11548,11503],"tags":[12693,12689,11655,12691,12688],"powerkit_post_featured":[],"class_list":["post-324128","post","type-post","status-publish","format-standard","has-post-thumbnail","category-education","category-endocrinology-and-diabetology","category-general-internal-medicine","category-partner-content-en","category-rx-en","category-studies","tag-antibody-determination","tag-autoimmune-polyendocrine-syndrome","tag-diabetes-en","tag-genetic-mutations","tag-type-1","pmpro-has-access"],"acf":[],"publishpress_future_action":{"enabled":false,"date":"2026-04-16 15:28:15","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category","extraData":[]},"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"wpml_current_locale":"en_US","wpml_translations":{"fr_FR":{"locale":"fr_FR","id":324137,"slug":"existe-t-il-des-signes-dun-syndrome-polyendocrinien-auto-immun","post_title":"Existe-t-il des signes d'un syndrome polyendocrinien auto-immun ?","href":"https:\/\/medizinonline.com\/fr\/existe-t-il-des-signes-dun-syndrome-polyendocrinien-auto-immun\/"},"it_IT":{"locale":"it_IT","id":324134,"slug":"ci-sono-segni-di-sindrome-poliendocrina-autoimmune","post_title":"Ci sono segni di sindrome poliendocrina autoimmune?","href":"https:\/\/medizinonline.com\/it\/ci-sono-segni-di-sindrome-poliendocrina-autoimmune\/"},"pt_PT":{"locale":"pt_PT","id":324143,"slug":"existem-sinais-de-sindrome-de-poliendocrina-auto-imune","post_title":"Existem sinais de s\u00edndrome de poliend\u00f3crina auto-imune?","href":"https:\/\/medizinonline.com\/pt-pt\/existem-sinais-de-sindrome-de-poliendocrina-auto-imune\/"},"es_ES":{"locale":"es_ES","id":324133,"slug":"existen-signos-de-sindrome-polendocrino-autoinmune","post_title":"\u00bfExisten signos de s\u00edndrome polendocrino autoinmune?","href":"https:\/\/medizinonline.com\/es\/existen-signos-de-sindrome-polendocrino-autoinmune\/"}},"_links":{"self":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts\/324128","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/comments?post=324128"}],"version-history":[{"count":0,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/posts\/324128\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/media\/126542"}],"wp:attachment":[{"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/media?parent=324128"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/category?post=324128"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/tags?post=324128"},{"taxonomy":"powerkit_post_featured","embeddable":true,"href":"https:\/\/medizinonline.com\/en\/wp-json\/wp\/v2\/powerkit_post_featured?post=324128"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}