{"id":6686,"date":"2020-04-22T18:14:30","date_gmt":"2020-04-22T21:14:30","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/sindrome-poliglandular-autoinmune-tipo-1-2\/"},"modified":"2020-04-27T17:08:33","modified_gmt":"2020-04-27T20:08:33","slug":"polyglandular-autoimmune-syndrome-type-1-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/","title":{"rendered":"Autoimmune polyglandular syndrome type 1"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Contents<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#Que-es-autoinmune-poliglandular-sindrome-%C2%BFtipo-1\" >What is autoimmune polyglandular syndrome type 1?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFQuien-contrae-el-sindrome-poliglandular-autoinmune-tipo-1\" >Who gets autoimmune polyglandular syndrome type 1?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFQue-causa-el-sindrome-poliglandular-autoinmune-tipo-1\" >What causes autoimmune polyglandular syndrome type 1?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFCuales-son-las-caracteristicas-clinicas-del-sindrome-poliglandular-autoinmune-tipo-1\" >What are the clinical characteristics of autoimmune polyglandular syndrome type 1?<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#Signos-cutaneos-de-APS-tipo-1\" >Skin signs of APS type 1<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFComo-se-diagnostica-el-sindrome-poliglandular-autoinmune-tipo-1\" >How is autoimmune polyglandular syndrome type 1 diagnosed?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFComo-se-trata-el-sindrome-poliglandular-autoinmune-tipo-1\" >How is autoimmune polyglandular syndrome type 1 treated?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-poliglandular-autoinmune-tipo-1-2\/#%C2%BFCual-es-el-resultado-del-sindrome-poliglandular-autoinmune-tipo-1\" >What is the outcome of autoimmune polyglandular syndrome type 1?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Que-es-autoinmune-poliglandular-sindrome-%C2%BFtipo-1\"><\/span>What is it <span class=\"term\" data-term-id=\"691\">autoimmune<\/span> polyglandular <span class=\"term\" data-term-id=\"350\">syndrome<\/span> Type 1?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Autoimmune polyglandular syndrome type 1 (APS1) is an autoimmune condition that causes multiple insufficiencies <span class=\"term\" data-term-id=\"1196\">endocrine<\/span> glands It is also known as autoimmune polyendocrine syndrome type 1, polyendocrinopathy-candidiasis.<span class=\"term\" data-term-id=\"375\">ectodermal<\/span> <span class=\"term\" data-term-id=\"74\">dystrophy<\/span> (APECED), Whitaker syndrome, and candidiasis-hypoparathyroidism-Addison&#039;s disease syndrome, among its many other names.<\/p>\n<p>APS1 was first described by Dr. Thomas Addison in the 19th century.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-contrae-el-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>Who gets autoimmune polyglandular syndrome type 1?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>APS1 is inherited, and women and girls are slightly more likely to develop the syndrome than men and boys. It occurs more frequently in particular ethnic populations due to <span class=\"term\" data-term-id=\"835\">consanguinity<\/span> or the grouping of descendants of a common family founder. It&#039;s more <span class=\"term\" data-term-id=\"1676\">predominant<\/span> in:<\/p>\n<ul>\n<li>Iranian Jews (1 in 9,000)<\/li>\n<li>Sardinians (1 in 14,400)<\/li>\n<li>Finns (1 in 25,000).<\/li>\n<\/ul>\n<p>APS1 is rare in other populations.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-causa-el-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>What causes autoimmune polyglandular syndrome type 1?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>APS1 is caused by <span class=\"term\" data-term-id=\"774\">gene<\/span> <span class=\"term\" data-term-id=\"871\">mutations<\/span> in the autoimmune regulatory gene, <em>AIR<\/em>, in <span class=\"term\" data-term-id=\"1271\">chromosome<\/span> 21q22. It is inherited in a <span class=\"term\" data-term-id=\"828\">autosomal<\/span> recessive pattern (two copies of an abnormal gene must be present for the syndrome to develop). These genetic mutations lead to <span class=\"term\" data-term-id=\"352\">autoantibodies<\/span> and cause <span class=\"term\" data-term-id=\"319\">chronic<\/span> <span class=\"term\" data-term-id=\"490\">inflammatory<\/span> cell <span class=\"term\" data-term-id=\"542\">infiltrators<\/span> in the affected organs<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-las-caracteristicas-clinicas-del-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>What are the clinical characteristics of autoimmune polyglandular syndrome type 1?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Symptoms appear most frequently in children aged 3 to 5 years, with most cases of APS1 appearing in early adolescence, and all cases by the time an individual is in their 30s.<\/p>\n<p>APS1 is based on three main clinical characteristics:<\/p>\n<ol>\n<li>\n<span class=\"term\" data-term-id=\"1019\">Mucocutaneous<\/span> candidiasis that affects the skin and <span class=\"term\" data-term-id=\"188\">mucous<\/span> membranes<\/li>\n<li>Hypoparathyroidism, which causes numbness and tingling in the face and extremities, muscle cramps and pain, weakness and fatigue due to low levels of circulating calcium.<\/li>\n<li>\n<p>Addison&#039;s disease, an insufficiency of the adrenal glands, which presents with skin changes. <span class=\"term\" data-term-id=\"1521\">pigmentation<\/span>, loss of appetite and weight loss, fatigue, low blood pressure and fatigue.<\/li>\n<\/ol>\n<p>While less common, other possible features of this syndrome may include:<\/p>\n<ul>\n<ul>\n<li>Hypogonadotropic <span class=\"term\" data-term-id=\"1840\">hypogonadism<\/span>\n<\/li>\n<li>Pernicious <span class=\"term\" data-term-id=\"598\">anemia<\/span>\n<\/li>\n<li>Chronic active <span class=\"term\" data-term-id=\"1801\">hepatitis<\/span>\n<\/li>\n<li>Asplenia<\/li>\n<li>Keratoconjunctivitis<\/li>\n<li>\n<span class=\"term\" data-term-id=\"750\">Interstitial<\/span> <span class=\"term\" data-term-id=\"994\">nephritis<\/span>\n<\/li>\n<li>\n<p>Diabetes mellitus type 1<\/li>\n<li>Cholelithiasis<\/li>\n<li><span class=\"term\" data-term-id=\"824\">Alopecia<\/span> areata<\/li>\n<li>Malabsorption<\/li>\n<li>\n<p>Vitiligo<\/li>\n<\/ul>\n<\/ul>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Signos-cutaneos-de-APS-tipo-1\"><\/span>Skin signs of APS type 1<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='vitiligo-09__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-8551146-6281668' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/vitiligo-09__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-8551146.jpg'>\n<\/div>\n<p class=\"p-small\">Vitiligo<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='alopecia-areata-1__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-9845921-9450009' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/alopecia-areata-1__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-9845921.jpg'>\n<\/div>\n<p class=\"p-small\">Alopecia areata<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='addison-01__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildizxq-7782008-7821300' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/addison-01__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDIzXQ-7782008.jpg'>\n<\/div>\n<p class=\"p-small\">Addison&#039;s disease<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-diagnostica-el-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>How is autoimmune polyglandular syndrome type 1 diagnosed?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>If an individual has evidence of more than one endocrine deficiency, further testing may be done to confirm autoimmune polyglandular syndrome type 1 (APS1), including:<\/p>\n<ul>\n<li>A <span class=\"term\" data-term-id=\"1430\">serum<\/span> autoimmune screen<\/li>\n<li>End organ function tests.<\/li>\n<\/ul>\n<p>Possible additional blood tests may include tests for <span class=\"term\" data-term-id=\"567\">testosterone<\/span>estradiol <span class=\"term\" data-term-id=\"1300\">follicle<\/span>stimulating hormone (FSH), luteinizing hormone (LH), prolactin, adrenocorticotropic hormone (ACTH), <span class=\"term\" data-term-id=\"1513\">plasma<\/span> renin activity, electrolyte levels, and a complete blood count. <\/p>\n<p>Swabs and skin scrapings may be taken to detect <em>Candida albicans<\/em>.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-trata-el-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>How is autoimmune polyglandular syndrome type 1 treated?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The treatment of APS1 will depend on its specific characteristics.<\/p>\n<ul>\n<li>\n<p>Mucocutaneous candidiasis is treated with oral antifungal agents, such as fluconazole or itraconazole.<\/li>\n<li>Hypoparathyroidism is treated with a combination of oral calcium and vitamin D (usually calcitriol).<\/li>\n<li>\n<p>Addison&#039;s disease is treated with oral corticosteroids and mineralosteroids. Adrenal gland transplants may also be indicated.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-resultado-del-sindrome-poliglandular-autoinmune-tipo-1\"><\/span>What is the outcome of autoimmune polyglandular syndrome type 1?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>the <span class=\"term\" data-term-id=\"241\">forecast<\/span> for APS1 it is variable. Survival rates have greatly improved since the 1970s.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>Que es autoinmune poliglandular s\u00edndrome \u00bftipo 1? El s\u00edndrome poliglandular autoinmune tipo 1 (APS1) es una afecci\u00f3n autoinmune que produce insuficiencias m\u00faltiples endocrino gl\u00e1ndulas Tambi\u00e9n se conoce como s\u00edndrome poliendocrino&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-6686","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/6686","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=6686"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/6686\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=6686"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=6686"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=6686"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}