{"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-auto-inflammatoires","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/fr\/wiki\/sindromes-autoinflamatorios\/","title":{"rendered":"Syndromes auto-inflammatoires."},"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\">Contenu<\/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=\"Basculer la table des mati\u00e8res\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Basculer<\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#Que-son-autoinflamatorio-sindromes\" >Quels sont les syndromes auto-inflammatoires?<\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#Clasificacion-de-los-sindromes-autoinflamatorios\" >Classification des syndromes auto-inflammatoires.<\/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\/fr\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#Otros-sindromes-autoinflamatorios-monogenicos\" >Autres syndromes auto-inflammatoires monog\u00e9niques<\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#Trastornos-no-hereditarios-o-poligenicos\" >Troubles non h\u00e9r\u00e9ditaires ou polyg\u00e9niques.<\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#Sistema-inmune-innato\" >Syst\u00e8me immunitaire inn\u00e9<\/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\/fr\/wiki\/sindromes-autoinflamatorios\/#%C2%BFCual-es-el-tratamiento-para-los-sindromes-autoinflamatorios\" >Quel est le traitement des syndromes auto-inflammatoires?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Que-son-autoinflamatorio-sindromes\"><\/span>Qui sont <span class=\"term\" data-term-id=\"1251\">auto-inflammatoire<\/span> syndromes?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Les syndromes auto-inflammatoires sont d\u00e9finis comme des conditions caus\u00e9es par une r\u00e9ponse exag\u00e9r\u00e9e du syst\u00e8me immunitaire inn\u00e9 entra\u00eenant des \u00e9pisodes spontan\u00e9s <span class=\"term\" data-term-id=\"148\">inflammation<\/span> affectant plusieurs organes. Un auto-inflammatoire <span class=\"term\" data-term-id=\"350\">syndrome<\/span> ne peut \u00eatre diagnostiqu\u00e9 qu&#039;en cas de maladie infectieuse, <span class=\"term\" data-term-id=\"1742\">malignit\u00e9<\/span>, les conditions allergiques et d&#039;immunod\u00e9ficience ont \u00e9t\u00e9 exclues. Compar\u00e9 au classique <span class=\"term\" data-term-id=\"691\">auto-immune<\/span> les maladies, les syndromes auto-inflammatoires font d\u00e9faut <span class=\"term\" data-term-id=\"1417\">agent pathog\u00e8ne<\/span> <span class=\"term\" data-term-id=\"352\">autoanticorps<\/span> et <span class=\"term\" data-term-id=\"410\">antig\u00e8ne<\/span>-sp\u00e9cifique <span class=\"term\" data-term-id=\"417\">Cellules T<\/span>.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"Clasificacion-de-los-sindromes-autoinflamatorios\"><\/span>Classification des syndromes auto-inflammatoires.<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Les syndromes auto-inflammatoires peuvent \u00eatre h\u00e9rit\u00e9s par <span class=\"term\" data-term-id=\"871\">mutations<\/span> \u00e0 une <span class=\"term\" data-term-id=\"774\">g\u00e8ne<\/span> (<span class=\"term\" data-term-id=\"1573\">monog\u00e9nique<\/span> syndromes auto-inflammatoires) ou, plus commun\u00e9ment, ce sont des conditions immunitaires polyg\u00e9niques qui ressemblent \u00e0 des auto-immunes <span class=\"term\" data-term-id=\"39\">collag\u00e8ne<\/span> troubles Le nombre de conditions incluses augmente \u00e0 mesure que <span class=\"term\" data-term-id=\"1851\">mol\u00e9culaire<\/span> et <span class=\"term\" data-term-id=\"558\">g\u00e9n\u00e9tique<\/span> Des \u00e9tudes r\u00e9v\u00e8lent les m\u00e9canismes de la maladie.<\/p>\n<p>Ensuite, un syst\u00e8me de classification est pr\u00e9sent\u00e9, avec des exemples de syndromes avec manifestations dermatologiques.<\/p>\n<h3><span class=\"ez-toc-section\" id=\"Hereditario-fiebre-sindromes\"><\/span>\n<span class=\"term\" data-term-id=\"1745\">H\u00e9r\u00e9ditaire<\/span> <span class=\"term\" data-term-id=\"1176\">fi\u00e8vre<\/span> syndromes<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li><span class=\"term\" data-term-id=\"849\">Famille<\/span> Fi\u00e8vre m\u00e9diterran\u00e9enne (FMF)<\/li>\n<li><span class=\"term\" data-term-id=\"289\">Tumeur<\/span> <span class=\"term\" data-term-id=\"403\">n\u00e9crose<\/span> facteur <span class=\"term\" data-term-id=\"782\">destinataire<\/span>syndrome de fi\u00e8vre p\u00e9riodique associ\u00e9 (TRAPS)<\/li>\n<li>Syndrome hyper-IgD (HIDS)<\/li>\n<\/ul>\n<h3><span class=\"ez-toc-section\" id=\"Otros-sindromes-autoinflamatorios-monogenicos\"><\/span>Autres syndromes auto-inflammatoires monog\u00e9niques<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li>\n<p>Syndromes p\u00e9riodiques associ\u00e9s \u00e0 la cryopyrine (CAPS) <\/p>\n<ul>\n<li>Syndrome familial auto-inflammatoire froid (FCAS)<\/li>\n<li>Syndrome de Muckle-Wells (MWS)<\/li>\n<li><span class=\"term\" data-term-id=\"1195\">N\u00e9onatal<\/span> d\u00e9marrage multi-syst\u00e8me <span class=\"term\" data-term-id=\"490\">inflammatoire<\/span> maladie\/<span class=\"term\" data-term-id=\"319\">chronique<\/span> Neurologique des enfants <span class=\"term\" data-term-id=\"47\">cutan\u00e9<\/span> <span class=\"term\" data-term-id=\"1576\">arthropathie<\/span> syndrome (NOMID \/ CINCA)<\/li>\n<\/ul>\n<\/li>\n<li>Syndrome de <span class=\"term\" data-term-id=\"254\">pyog\u00e8ne<\/span> <span class=\"term\" data-term-id=\"1578\">arthrite<\/span>, <span class=\"term\" data-term-id=\"1626\">pyoderma<\/span> gangreneuse et acn\u00e9 (syndrome PAPA, PAPAS, syndrome PAPGA)<\/li>\n<li><span class=\"term\" data-term-id=\"1610\">Jeunesse<\/span> <span class=\"term\" data-term-id=\"380\">syst\u00e9mique<\/span> granulomatose (syndrome de Blau, sarco\u00efdose pr\u00e9coce)<\/li>\n<li>Carence <span class=\"term\" data-term-id=\"915\">interleukine<\/span>-1 antagoniste des r\u00e9cepteurs (DIRA)<\/li>\n<li>Acidurie m\u00e9valonique<\/li>\n<li>Syndrome de Majeed<\/li>\n<\/ul>\n<h3><span class=\"ez-toc-section\" id=\"Trastornos-no-hereditarios-o-poligenicos\"><\/span>Troubles non h\u00e9r\u00e9ditaires ou polyg\u00e9niques.<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<ul>\n<li>Syndrome de Schnitzler<\/li>\n<li>la maladie de Crohn<\/li>\n<li>Maladie de Behcet<\/li>\n<li>  Hidrosad\u00e9nite <\/li>\n<li>Arthrite psoriasique<\/li>\n<li>Fi\u00e8vre p\u00e9riodique, syndrome de la fi\u00e8vre aphteuse <span class=\"term\" data-term-id=\"272\">stomatite<\/span>, <span class=\"term\" data-term-id=\"1223\">pharyngite<\/span> et ad\u00e9nite (PAPAS, syndrome PFAPA)<\/li>\n<li>Juv\u00e9nile \u00e0 d\u00e9but syst\u00e9mique <span class=\"term\" data-term-id=\"862\">idiopathique<\/span> arthrite<\/li>\n<li>Maladie de l&#039;adulte<\/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>Syst\u00e8me immunitaire inn\u00e9<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Le syst\u00e8me immunitaire inn\u00e9 est un syst\u00e8me h\u00e9r\u00e9ditaire primitif pour reconna\u00eetre le danger sous la forme d&#039;une blessure ou <span class=\"term\" data-term-id=\"145\">infection<\/span>. <span class=\"term\" data-term-id=\"457\">Macrophages<\/span> et <span class=\"term\" data-term-id=\"51\">cellules dendritiques<\/span> transporter des r\u00e9cepteurs qui se lient \u00e0 <span class=\"term\" data-term-id=\"586\">agent pathog\u00e8ne<\/span>les \u00e9talons mol\u00e9culaires associ\u00e9s (PAMP), qui sont des composants de la paroi cellulaire microbienne qui sont constamment exprim\u00e9s par <span class=\"term\" data-term-id=\"935\">les bact\u00e9ries<\/span> et les virus, ou mod\u00e8les mol\u00e9culaires associ\u00e9s au danger (DAMP), qui sont produits par le corps en r\u00e9ponse \u00e0 une blessure ou une infection. Les r\u00e9cepteurs de reconnaissance de formes sont h\u00e9rit\u00e9s et ne s&#039;adaptent pas ou ne changent pas avec l&#039;exp\u00e9rience. La liaison de ces r\u00e9cepteurs coordonne une r\u00e9ponse inflammatoire initiale qui implique <span class=\"term\" data-term-id=\"196\"><span class=\"term\" data-term-id=\"442\">neutrophiles<\/span><\/span>  et <span class=\"term\" data-term-id=\"454\">monocytes<\/span> et la production de <span class=\"term\" data-term-id=\"434\">cytokines<\/span> comme l&#039;interleukine 1 (IL-1). IL-1 est activ\u00e9 dans le <span class=\"term\" data-term-id=\"838\">cytoplasme<\/span> des neutrophiles et des monocytes par les inflammasomes, grands complexes prot\u00e9iques qui incluent l&#039;activation <span class=\"term\" data-term-id=\"1525\">enzyme<\/span> caspase-1. L&#039;IL-1 activ\u00e9e est le d\u00e9clencheur le plus puissant connu pour provoquer de la fi\u00e8vre. Il est \u00e9galement activ\u00e9 <span class=\"term\" data-term-id=\"421\">lymphocytes<\/span> et favorise les globules blancs <span class=\"term\" data-term-id=\"543\">infiltration<\/span> sur les sites de blessure ou d&#039;infection.<\/p>\n<p>Des mutations g\u00e9n\u00e9tiques affectant les composants de l&#039;inflammasome ou la r\u00e9ponse inflammatoire activ\u00e9e par IL-1 ont \u00e9t\u00e9 trouv\u00e9es dans plusieurs des syndromes auto-inflammatoires monog\u00e9niques. Les mutations peuvent provoquer une hyperactivit\u00e9 de l&#039;inflammasome ou ne pas limiter l&#039;inflammation m\u00e9di\u00e9e par l&#039;IL-1. Les maladies auto-inflammatoires qui ne sont pas consid\u00e9r\u00e9es comme g\u00e9n\u00e9tiques sont associ\u00e9es \u00e0 des maladies h\u00e9r\u00e9ditaires. <span class=\"term\" data-term-id=\"929\">polymorphismes<\/span> des prot\u00e9ines comme la gamma-s\u00e9cr\u00e9tase et les prot\u00e9ines associ\u00e9es \u00e0 Notch, r\u00e9sultant en <span class=\"term\" data-term-id=\"1463\">d\u00e9r\u00e9glementation<\/span> inflammation lorsqu&#039;elle est expos\u00e9e \u00e0 certains d\u00e9clencheurs. Il s&#039;agit notamment des hormones, du tabagisme, des adipokines associ\u00e9es \u00e0 <span class=\"term\" data-term-id=\"1510\">insuline<\/span> r\u00e9sistance et ob\u00e9sit\u00e9.<\/p>\n<p>Dans les syndromes auto-inflammatoires, les voies effectrices sont hypersensibles \u00e0 <span class=\"term\" data-term-id=\"1096\">endog\u00e8ne<\/span> (DAMP) ou <span class=\"term\" data-term-id=\"1095\">exog\u00e8ne<\/span> (PAMP) mod\u00e8les mol\u00e9culaires, ou sont hyperactifs constitutivement, r\u00e9sultant en incontr\u00f4l\u00e9 <span class=\"term\" data-term-id=\"433\">cytokine<\/span>-inflammation m\u00e9di\u00e9e.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-para-los-sindromes-autoinflamatorios\"><\/span>Quel est le traitement des syndromes auto-inflammatoires?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Le traitement varie avec le syndrome r\u00e9el. \u00c0 bien des \u00e9gards, les corticost\u00e9ro\u00efdes syst\u00e9miques n&#039;ont qu&#039;un effet modeste. Des agents biologiques tels que l&#039;anakinra (qui cible l&#039;IL-1) entra\u00eenent une am\u00e9lioration spectaculaire et constante des syndromes o\u00f9 un lien clair avec l&#039;IL-1 a \u00e9t\u00e9 d\u00e9montr\u00e9. Il y a un avantage moins constant dans d&#039;autres conditions o\u00f9 un lien direct avec IL-1 n&#039;a pas \u00e9t\u00e9 trouv\u00e9.<\/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\/fr\/wp-json\/wp\/v2\/wiki\/7409","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/comments?post=7409"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/wiki\/7409\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/media?parent=7409"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/categories?post=7409"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/tags?post=7409"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}