{"id":7409,"date":"2020-04-24T09:15:07","date_gmt":"2020-04-24T12:15:07","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/sindromes-autoinflamatorios\/"},"modified":"2020-04-27T16:24:38","modified_gmt":"2020-04-27T19:24:38","slug":"syndromes-autoinflammatory-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/sindromes-autoinflamatorios\/","title":{"rendered":"Autoinflammatory syndromes."},"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\/sindromes-autoinflamatorios\/#Que-son-autoinflamatorio-sindromes\" >What are autoinflammatory syndromes?<\/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\/sindromes-autoinflamatorios\/#Clasificacion-de-los-sindromes-autoinflamatorios\" >Classification of autoinflammatory syndromes.<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindromes-autoinflamatorios\/#Hereditario-fiebre-sindromes\" >Hereditario fiebre s\u00edndromes<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindromes-autoinflamatorios\/#Otros-sindromes-autoinflamatorios-monogenicos\" >Other monogenic autoinflammatory syndromes<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindromes-autoinflamatorios\/#Trastornos-no-hereditarios-o-poligenicos\" >Non-hereditary or polygenic disorders.<\/a><\/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\/sindromes-autoinflamatorios\/#Sistema-inmune-innato\" >Innate immune system<\/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\/sindromes-autoinflamatorios\/#%C2%BFCual-es-el-tratamiento-para-los-sindromes-autoinflamatorios\" >What is the treatment for autoinflammatory syndromes?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Que-son-autoinflamatorio-sindromes\"><\/span>What are they <span class=\"term\" data-term-id=\"1251\">autoinflammatory<\/span> syndromes?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Autoinflammatory syndromes are defined as conditions caused by an exaggerated response of the innate immune system that results in spontaneous episodes <span class=\"term\" data-term-id=\"148\">inflammation<\/span> affecting multiple organs. An autoinflammatory <span class=\"term\" data-term-id=\"350\">syndrome<\/span> can only be diagnosed when there are infectious conditions, <span class=\"term\" data-term-id=\"1742\">malignancy<\/span>, allergic and immunodeficiency conditions have been excluded. Compared to the classic <span class=\"term\" data-term-id=\"691\">autoimmune<\/span> diseases, autoinflammatory syndromes lack <span class=\"term\" data-term-id=\"1417\">pathogen<\/span> <span class=\"term\" data-term-id=\"352\">autoantibodies<\/span> and <span class=\"term\" data-term-id=\"410\">antigen<\/span>-specific <span class=\"term\" data-term-id=\"417\">T cells<\/span>.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"Clasificacion-de-los-sindromes-autoinflamatorios\"><\/span>Classification of autoinflammatory syndromes.<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Autoinflammatory syndromes can be inherited through <span class=\"term\" data-term-id=\"871\">mutations<\/span> to a single <span class=\"term\" data-term-id=\"774\">gene<\/span> (<span class=\"term\" data-term-id=\"1573\">monogenic<\/span> autoinflammatory syndromes) or, more commonly, are polygenic immune conditions that resemble autoimmune <span class=\"term\" data-term-id=\"39\">collagen<\/span> disorders The number of conditions included increases as <span class=\"term\" data-term-id=\"1851\">molecular<\/span> and <span class=\"term\" data-term-id=\"558\">genetic<\/span> Studies reveal the mechanisms of the disease.<\/p>\n<p>A classification system is presented below, with examples of syndromes with dermatological manifestations.<\/p>\n<h3><span class=\"ez-toc-section\" id=\"Hereditario-fiebre-sindromes\"><\/span>\n<span class=\"term\" data-term-id=\"1745\">Hereditary<\/span> <span class=\"term\" data-term-id=\"1176\">fever<\/span> syndromes<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li><span class=\"term\" data-term-id=\"849\">Family<\/span> Mediterranean fever (FMF)<\/li>\n<li><span class=\"term\" data-term-id=\"289\">Tumor<\/span> <span class=\"term\" data-term-id=\"403\">necrosis<\/span> factor <span class=\"term\" data-term-id=\"782\">receiver<\/span>associated periodic fever syndrome (TRAPS)<\/li>\n<li>Hyper-IgD syndrome (HIDS)<\/li>\n<\/ul>\n<h3><span class=\"ez-toc-section\" id=\"Otros-sindromes-autoinflamatorios-monogenicos\"><\/span>Other monogenic autoinflammatory syndromes<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li>\n<p>Cryopyrin-associated periodic syndromes (CAPS) <\/p>\n<ul>\n<li>Familial cold autoinflammatory syndrome (FCAS)<\/li>\n<li>Muckle-Wells syndrome (MWS)<\/li>\n<li><span class=\"term\" data-term-id=\"1195\">Neonatal<\/span> multi-system startup <span class=\"term\" data-term-id=\"490\">inflammatory<\/span> disease\/<span class=\"term\" data-term-id=\"319\">chronic<\/span> Child neurological <span class=\"term\" data-term-id=\"47\">cutaneous<\/span> <span class=\"term\" data-term-id=\"1576\">arthropathy<\/span> syndrome (NOMID \/ CINCA)<\/li>\n<\/ul>\n<\/li>\n<li>Syndrome of <span class=\"term\" data-term-id=\"254\">pyogenic<\/span> <span class=\"term\" data-term-id=\"1578\">arthritis<\/span>, <span class=\"term\" data-term-id=\"1626\">pyoderma<\/span> gangrenosum and acne (PAPA syndrome, PAPAS, PAPGA syndrome)<\/li>\n<li><span class=\"term\" data-term-id=\"1610\">Youth<\/span> <span class=\"term\" data-term-id=\"380\">systemic<\/span> granulomatosis (Blau syndrome, early-onset sarcoidosis)<\/li>\n<li>Deficiency of <span class=\"term\" data-term-id=\"915\">interleukin<\/span>-1 receptor antagonist (DIRA)<\/li>\n<li>Mevalonic aciduria<\/li>\n<li>Majeed syndrome<\/li>\n<\/ul>\n<h3><span class=\"ez-toc-section\" id=\"Trastornos-no-hereditarios-o-poligenicos\"><\/span>Non-hereditary or polygenic disorders.<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li>Schnitzler&#039;s syndrome<\/li>\n<li>Crohn&#039;s disease<\/li>\n<li>Behcet&#039;s disease<\/li>\n<li>  Hidradenitis suppurativa <\/li>\n<li>Psoriasic arthritis<\/li>\n<li>Periodic fever syndrome, foot and mouth <span class=\"term\" data-term-id=\"272\">stomatitis<\/span>, <span class=\"term\" data-term-id=\"1223\">pharyngitis<\/span> and adenitis (PAPAS, PFAPA syndrome)<\/li>\n<li>Juvenile systemic onset <span class=\"term\" data-term-id=\"862\">idiopathic<\/span> arthritis<\/li>\n<li>Adult-onset disease<\/li>\n<\/ul>\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=\"Sistema-inmune-innato\"><\/span>Innate immune system<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The innate immune system is a primitive inherited system to recognize danger in the form of injury or <span class=\"term\" data-term-id=\"145\">infection<\/span>. <span class=\"term\" data-term-id=\"457\">Macrophages<\/span> and <span class=\"term\" data-term-id=\"51\">dendritic cells<\/span> carry receivers that bind to <span class=\"term\" data-term-id=\"586\">pathogen<\/span>associated molecular patterns (PAMPs), which are components of the microbial cell wall that are consistently expressed by <span class=\"term\" data-term-id=\"935\">bacteria<\/span> and viruses, or hazard-associated molecular patterns (DAMPs), that are produced by the body in response to injury or infection. Pattern recognition receptors are inherited and do not adapt or change with experience. The binding of these receptors coordinates an initial inflammatory response that involves <span class=\"term\" data-term-id=\"196\"><span class=\"term\" data-term-id=\"442\">neutrophils<\/span><\/span>  and <span class=\"term\" data-term-id=\"454\">monocytes<\/span> and the production of <span class=\"term\" data-term-id=\"434\">cytokines<\/span> such as interleukin 1 (IL-1). IL-1 is activated within the <span class=\"term\" data-term-id=\"838\">cytoplasm<\/span> of neutrophils and monocytes by inflammasomes, large protein complexes that include the activation <span class=\"term\" data-term-id=\"1525\">enzyme<\/span> caspase-1. Activated IL-1 is the strongest known trigger for fever. Also activates <span class=\"term\" data-term-id=\"421\">lymphocytes<\/span> and promotes white blood cells <span class=\"term\" data-term-id=\"543\">infiltration<\/span> at sites of injury or infection.<\/p>\n<p>Genetic mutations affecting components of the inflammasome or the inflammatory response activated by IL-1 have been found in several of the monogenic autoinflammatory syndromes. Mutations can cause inflammation of the inflammasome or fail to limit IL-1-mediated inflammation. Autoinflammatory diseases that are not considered genetic are associated with inherited diseases. <span class=\"term\" data-term-id=\"929\">polymorphisms<\/span> of proteins such as gamma-secretase and Notch-associated proteins, resulting in <span class=\"term\" data-term-id=\"1463\">deregulation<\/span> of inflammasome when exposed to certain triggers. These include hormones, smoking, adipokines associated with <span class=\"term\" data-term-id=\"1510\">insulin<\/span> resistance and obesity.<\/p>\n<p>In autoinflammatory syndromes, the effector pathways are hypersensitive to <span class=\"term\" data-term-id=\"1096\">endogenous<\/span> (DAMP) or <span class=\"term\" data-term-id=\"1095\">exogenous<\/span> (PAMP) molecular patterns, or are constitutively hyperactive, resulting in uncontrolled <span class=\"term\" data-term-id=\"433\">cytokine<\/span>-mediated inflammation.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-para-los-sindromes-autoinflamatorios\"><\/span>What is the treatment for autoinflammatory syndromes?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment varies with the actual syndrome. In many ways, systemic corticosteroids have only a modest effect. Biological agents such as anakinra (which targets IL-1) result in dramatic and consistent improvement in syndromes where a clear link to IL-1 has been demonstrated. There is less consistent benefit in other conditions where a direct link to IL-1 has not been found.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>Qu\u00e9 son autoinflamatorio s\u00edndromes? Los s\u00edndromes autoinflamatorios se definen como afecciones causadas por una respuesta exagerada del sistema inmune innato que resulta en episodios espont\u00e1neos inflamaci\u00f3n afectando m\u00faltiples \u00f3rganos. Un&#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-7409","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7409","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=7409"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7409\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=7409"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=7409"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=7409"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}