{"id":1415,"date":"2026-07-10T15:42:36","date_gmt":"2026-07-10T13:42:36","guid":{"rendered":"https:\/\/amyloidosisalliance.local\/amylose-attrv\/"},"modified":"2026-09-29T11:03:54","modified_gmt":"2026-09-29T09:03:54","slug":"amylose-attrv","status":"publish","type":"page","link":"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/amylose-attrv\/","title":{"rendered":"Amylose ATTRv"},"content":{"rendered":"\n<nav aria-label=\"Breadcrumb\" class=\"wp-block-group aa-breadcrumb aa-events-hero__crumb is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"aa-inner\"><a href=\"\/\">Accueil<\/a><span class=\"aa-breadcrumb__sep\">\u203a<\/span><span class=\"aa-breadcrumb__label\">Amylose<\/span><span class=\"aa-breadcrumb__sep\">\u203a<\/span><a href=\"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/\">Types d\u2019amylose<\/a><span class=\"aa-breadcrumb__sep\">\u203a<\/span><a href=\"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/amylose-attr\/\">Amylose ATTR<\/a><span class=\"aa-breadcrumb__sep\">\u203a<\/span><span class=\"aa-breadcrumb__current\">ATTRv<\/span><\/div>\n<\/nav>\n\n<section class=\"wp-block-group aa-events-hero aa-events-hero--art aa-events-hero--attrv is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-events-hero__kicker wp-block-paragraph\">Amylose ATTR<\/p>\n\n\n\n<h1 class=\"wp-block-heading\">Amylose ATTRv<\/h1>\n\n\n\n<p class=\"wp-block-paragraph\">L\u2019amylose ATTR h\u00e9r\u00e9ditaire, aussi appel\u00e9e amylose ATTR variante (ATTRv), est due \u00e0 un variant pathog\u00e8ne du g\u00e8ne TTR, h\u00e9rit\u00e9 d\u2019un parent. C\u2019est la m\u00eame prot\u00e9ine que dans l\u2019amylose ATTRwt, mais d\u00e9stabilis\u00e9e d\u2019embl\u00e9e, et non par le seul effet de l\u2019\u00e2ge. Elle peut toucher les nerfs, le c\u0153ur, ou les deux, et comme elle se transmet dans les familles, le diagnostic ne concerne jamais une seule personne.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-events-hero__art is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-full\"><img decoding=\"async\" src=\"\/wp-content\/themes\/amyloidosis-alliance\/assets\/images\/disease\/attrv.svg\" alt=\"Sch&#xE9;ma&#xA0;: une transthyr&#xE9;tine variante se dissocie plus facilement, et ses sous-unit&#xE9;s se replient mal, s&#x2019;alignent et s&#x2019;empilent en une fibrille amylo&#xEF;de\"\/><\/figure>\n<\/div>\n<\/section>\n\n<nav aria-label=\"Section navigation\" class=\"wp-block-group aa-anchorband is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-columns aa-anchor-cols is-layout-flex wp-container-core-columns-is-layout-b2e659ba wp-block-columns-is-layout-flex\">\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">01<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#what\">Qu\u2019est-ce que l\u2019ATTRv\u202f?<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">02<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#forms\">Deux formes<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">03<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#symptoms\">Sympt\u00f4mes<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">04<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#diagnosis\">Diagnostic<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">05<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#treatment\">Traitement<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">06<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#family\">Famille<\/a><\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column aa-anchor-item is-layout-flow wp-block-column-is-layout-flow\">\n<p class=\"num wp-block-paragraph\">07<\/p>\n\n\n\n<p class=\"lbl wp-block-paragraph\"><a href=\"#follow-up\">Suivi<\/a><\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/nav>\n\n<section id=\"what\" class=\"wp-block-group aa-section aa-section--white aa-what is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">01<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Qu\u2019est-ce que l\u2019ATTRv\u202f?<\/h2>\n\n\n\n<div class=\"wp-block-group aa-key-idea is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-key-idea__label wp-block-paragraph\">\u00c0 retenir<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">La mutation vient d\u2019un des parents. Les sympt\u00f4mes peuvent appara\u00eetre \u00e0 tout \u00e2ge \u00e0 partir du d\u00e9but de l\u2019\u00e2ge adulte, et un m\u00eame variant se comporte diff\u00e9remment d\u2019une famille \u00e0 l\u2019autre.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-what-cols is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-what-col is-layout-flow wp-block-group-is-layout-flow\">\n<h4 class=\"wp-block-heading\">Une prot\u00e9ine mut\u00e9e<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">L\u2019ATTRv est due \u00e0 une mutation du <strong>g\u00e8ne TTR<\/strong>, h\u00e9rit\u00e9e d\u2019un parent. La transmission est autosomique dominante. La mutation fait que la transthyr\u00e9tine se replie anormalement\u202f; la prot\u00e9ine mal repli\u00e9e s\u2019agr\u00e8ge en fibrilles amylo\u00efdes, qui s\u2019accumulent au fil des ann\u00e9es.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Plus de 130 variants du g\u00e8ne sont connus. Certains sont li\u00e9s \u00e0 des r\u00e9gions et \u00e0 des communaut\u00e9s particuli\u00e8res\u00a0: au Royaume-Uni, V122I et T60A sont parmi les plus fr\u00e9quents.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-what-col is-layout-flow wp-block-group-is-layout-flow\">\n<h4 class=\"wp-block-heading\">Quand elle commence<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Les sympt\u00f4mes peuvent se d\u00e9clarer \u00e0 tout moment d\u00e8s le d\u00e9but de la vie adulte. L\u2019\u00e2ge auquel ils apparaissent d\u00e9pend du variant et de l\u2019origine familiale.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Dans la forme polyneuropathique, la maladie d\u00e9bute vers 30 ans chez les patients d\u2019origine portugaise, et vers 60 ans en moyenne chez les autres. Deux personnes porteuses de la m\u00eame mutation peuvent tomber malades \u00e0 plusieurs d\u00e9cennies d\u2019\u00e9cart.<\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/section>\n\n<section class=\"wp-block-group aa-section aa-section--white aa-h-facts-sec is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-facts is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-fact is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-fact__num wp-block-paragraph\">130+<\/p>\n\n\n\n<p class=\"aa-tp-fact__lbl wp-block-paragraph\">variants connus du g\u00e8ne TTR, avec d\u2019importantes diff\u00e9rences de r\u00e9partition g\u00e9ographique et de pr\u00e9sentation clinique.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-fact aa-tp-fact--text is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-fact__lbl wp-block-paragraph\">Le d\u00e9lai de diagnostic varie beaucoup selon les personnes, les pr\u00e9sentations cliniques et les syst\u00e8mes de sant\u00e9\u202f; reconna\u00eetre la maladie t\u00f4t reste un enjeu majeur dans le monde entier.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-fact aa-tp-fact--text is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-fact__lbl wp-block-paragraph\">L\u2019ATTRv est pr\u00e9sente dans le monde entier, mais sa pr\u00e9valence varie fortement selon le pays, l\u2019ascendance et le variant de la TTR, avec des zones d\u2019end\u00e9mie reconnues dans plusieurs r\u00e9gions.<\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"forms\" class=\"wp-block-group aa-section aa-section--cream aa-tp-duo-sec is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-centered-head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">02<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Deux formes, une maladie<\/h2>\n\n\n\n<div class=\"aa-divider-dot\"><\/div>\n\n\n\n<p class=\"wp-block-paragraph\">Le variant port\u00e9 d\u00e9termine, pour une large part, l\u2019endroit o\u00f9 se d\u00e9pose la substance amylo\u00efde. Les m\u00e9decins d\u00e9crivent deux tableaux cliniques, m\u00eame si de tr\u00e8s nombreux patients pr\u00e9sentent un peu des deux.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-duo is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-duo__card is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-duo__tag wp-block-paragraph\">ATTRv-PN<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Polyneuropathie amylo\u00efde h\u00e9r\u00e9ditaire<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Aussi appel\u00e9e polyneuropathie amylo\u00efde familiale. Ce sont les nerfs qui en paient le prix\u00a0: la sensibilit\u00e9 s\u2019estompe dans les pieds, les nerfs autonomes d\u00e9faillent, et la marche devient incertaine. Le c\u0153ur est aussi souvent touch\u00e9.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Sans traitement, elle est potentiellement tr\u00e8s grave et invalidante.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-duo__card is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-duo__tag wp-block-paragraph\">ATTRv-CM<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Cardiomyopathie amylo\u00efde familiale<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Une amylose syst\u00e9mique h\u00e9r\u00e9ditaire dans laquelle les manifestations cardiaques dominent. Les d\u00e9p\u00f4ts rigidifient et \u00e9paississent les parois des ventricules, r\u00e9duisant les cavit\u00e9s et affaiblissant le d\u00e9bit sanguin.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Un syndrome du canal carpien la pr\u00e9c\u00e8de souvent de cinq \u00e0 dix ans.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-note is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"wp-block-paragraph\">En pratique clinique, ces deux tableaux sont rarement aussi distincts que leurs noms le laissent penser. Chez de tr\u00e8s nombreux patients, les manifestations se chevauchent largement\u00a0: ce sont des formes mixtes, neurologiques et cardiaques.<\/p>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"symptoms\" class=\"wp-block-group aa-section aa-section--white aa-tp-symptoms is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-centered-head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">03<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Sympt\u00f4mes<\/h2>\n\n\n\n<div class=\"aa-divider-dot\"><\/div>\n\n\n\n<p class=\"wp-block-paragraph\">La maladie s\u2019annonce g\u00e9n\u00e9ralement dans les pieds, discr\u00e8tement, et on l\u2019attribue \u00e0 autre chose. Il s\u2019\u00e9coule en moyenne trois \u00e0 six ans avant qu\u2019elle soit nomm\u00e9e.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-organs aa-tp-organs--major is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-organ is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Nerfs et sensibilit\u00e9<\/h3>\n\n\n\n<p class=\"aa-tp-organ__tag wp-block-paragraph\">L\u00e0 o\u00f9 tout commence<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">La sensibilit\u00e9 \u00e0 la temp\u00e9rature et \u00e0 la douleur s\u2019estompe dans les pieds. Des br\u00fblures et des fourmillements surviennent sans raison. Des blessures passent inaper\u00e7ues parce qu\u2019on ne les sent pas. La faiblesse s\u2019\u00e9tend des pieds vers les mains, et la marche et l\u2019\u00e9quilibre en souffrent.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-organ is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Le syst\u00e8me nerveux autonome<\/h3>\n\n\n\n<p class=\"aa-tp-organ__tag wp-block-paragraph\">Les nerfs auxquels on ne pense jamais<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Naus\u00e9es, digestion lente, perte d\u2019app\u00e9tit, vomissements. Constipation alternant avec des diarrh\u00e9es. Troubles de l\u2019\u00e9rection. Vertiges ou malaises en se levant. Troubles urinaires. Perte de poids involontaire de plus de cinq kilos en six mois.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-organ is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Le c\u0153ur<\/h3>\n\n\n\n<p class=\"aa-tp-organ__tag wp-block-paragraph\">Un patient sur trois a besoin d\u2019un pacemaker<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">La conduction ralentit, parfois jusqu\u2019\u00e0 une bradycardie extr\u00eame, avec un risque d\u2019arr\u00eat cardiaque\u00a0: environ un patient sur trois a besoin d\u2019un pacemaker. La substance amylo\u00efde infiltre le muscle lui-m\u00eame, et l\u2019insuffisance cardiaque provoque un essoufflement \u00e0 l\u2019effort.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-organs aa-tp-organs--minor is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-organ is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Les yeux<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Des d\u00e9p\u00f4ts dans le vitr\u00e9, observ\u00e9s chez 20 \u00e0 30 % des patients, apparaissent comme des corps flottants qui traversent le champ de vision. Un glaucome touche environ 20 % des patients. La s\u00e9cheresse oculaire, avec une sensation de sable dans les yeux, est fr\u00e9quente.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-organ is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Syndrome du canal carpien<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Dans la forme cardiaque, il pr\u00e9c\u00e8de les sympt\u00f4mes cardiaques de cinq \u00e0 dix ans, d\u2019une main ou des deux. C\u2019est souvent le tout premier signe de la maladie.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-flags is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-flags__photo is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"683\" src=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-anatomical-heart-model-resting-on-a-page-of-ecg-traces-1024x683.jpg\" alt=\"Un mod&#xE8;le anatomique de c&#x153;ur pos&#xE9; sur une page de trac&#xE9;s d&#x2019;ECG\" class=\"wp-image-692\" srcset=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-anatomical-heart-model-resting-on-a-page-of-ecg-traces-1024x683.jpg 1024w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-anatomical-heart-model-resting-on-a-page-of-ecg-traces-300x200.jpg 300w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-anatomical-heart-model-resting-on-a-page-of-ecg-traces-768x512.jpg 768w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-anatomical-heart-model-resting-on-a-page-of-ecg-traces-1536x1024.jpg 1536w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-flags__body is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Quand consulter<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Ces signes comptent tout particuli\u00e8rement si une amylose est d\u00e9j\u00e0 connue dans votre famille. Dites-le \u00e0 votre m\u00e9decin\u00a0: cela change ce qu\u2019il recherche.<\/p>\n\n\n\n<ul class=\"wp-block-list aa-tp-flags__list\">\n<li>Engourdissements ou br\u00fblures dans les pieds<\/li>\n\n\n\n<li>Des blessures aux pieds qui passent inaper\u00e7ues<\/li>\n\n\n\n<li>Une marche instable, une perte d\u2019\u00e9quilibre<\/li>\n\n\n\n<li>Naus\u00e9es, vomissements, sensation de sati\u00e9t\u00e9 trop rapide<\/li>\n\n\n\n<li>Constipation alternant avec des diarrh\u00e9es<\/li>\n\n\n\n<li>Vertiges ou malaises en se levant<\/li>\n\n\n\n<li>Troubles de l\u2019\u00e9rection<\/li>\n\n\n\n<li>Troubles urinaires<\/li>\n\n\n\n<li>Perte de plus de cinq kilos en six mois<\/li>\n\n\n\n<li>Des corps flottants qui traversent le champ de vision<\/li>\n<\/ul>\n\n\n\n<div class=\"wp-block-group aa-tp-alert is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"wp-block-paragraph\">L\u2019ATTRv se transmet dans les familles. Un diagnostic chez une personne est une question pos\u00e9e \u00e0 tous ses apparent\u00e9s.<\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"diagnosis\" class=\"wp-block-group aa-section aa-section--cream aa-tp-dx is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-dx__body is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">04<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Comment l\u2019ATTRv est diagnostiqu\u00e9e<\/h2>\n\n\n\n<p class=\"aa-tp-dx__intro wp-block-paragraph\">Un seul examen tranche la question, et c\u2019est une prise de sang. La difficult\u00e9 se situe en amont\u00a0: une neuropathie qui ressemble \u00e0 cent autres, et qu\u2019on leur attribue pendant trois \u00e0 six ans.<\/p>\n\n\n\n<div class=\"wp-block-group aa-tp-dx__steps is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-dx__step is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-dx__step-num wp-block-paragraph\">01<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Consulter un sp\u00e9cialiste<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Un neurologue pour la forme polyneuropathique, un cardiologue pour la forme cardiaque. Les sympt\u00f4mes sont volontiers attribu\u00e9s \u00e0 des causes plus fr\u00e9quentes de neuropathie, et le d\u00e9lai avant le diagnostic est en moyenne de trois \u00e0 six ans.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-dx__step is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-dx__step-num wp-block-paragraph\">02<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Un test g\u00e9n\u00e9tique sanguin<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Il confirme la mutation du g\u00e8ne TTR. C\u2019est l\u2019examen qui nomme la maladie, et celui qui distingue l\u2019amylose ATTRwt de la forme h\u00e9r\u00e9ditaire.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-dx__step is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-dx__step-num wp-block-paragraph\">03<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Mettre en \u00e9vidence la substance amylo\u00efde<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Une biopsie montre les d\u00e9p\u00f4ts. Pour le c\u0153ur, l\u2019\u00e9chocardiographie ou l\u2019IRM cardiaque r\u00e9v\u00e8lent l\u2019\u00e9paississement des parois, et la scintigraphie osseuse \u00e9taye le diagnostic sans biopsie. L\u2019amylose AL doit \u00eatre \u00e9cart\u00e9e.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-dx__step is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-tp-dx__step-num wp-block-paragraph\">04<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">D\u00e9pister la famille<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Une fois une mutation identifi\u00e9e, un test peut \u00eatre propos\u00e9 aux apparent\u00e9s. Dans la forme cardiaque, cela conduit \u00e0 un bilan syst\u00e9matique de la famille, pour trouver la maladie avant qu\u2019elle ne se manifeste.<\/p>\n<\/div>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-dx__photo is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"683\" src=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/a-researcher-wearing-protective-goggles-in-a-laboratory-1024x683.jpg\" alt=\"Une chercheuse portant des lunettes de protection dans un laboratoire\" class=\"wp-image-693\" srcset=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/a-researcher-wearing-protective-goggles-in-a-laboratory-1024x683.jpg 1024w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/a-researcher-wearing-protective-goggles-in-a-laboratory-300x200.jpg 300w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/a-researcher-wearing-protective-goggles-in-a-laboratory-768x512.jpg 768w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/a-researcher-wearing-protective-goggles-in-a-laboratory-1536x1024.jpg 1536w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"treatment\" class=\"wp-block-group aa-section aa-section--white aa-treating is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-centered-head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">05<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Traitement<\/h2>\n\n\n\n<div class=\"aa-divider-dot\"><\/div>\n\n\n\n<p class=\"wp-block-paragraph\">Plusieurs m\u00e9dicaments ralentissent d\u00e9sormais cette maladie. Aucun ne r\u00e9pare ce qu\u2019elle a d\u00e9j\u00e0 fait.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-treat-lead is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-treat-lead__photo is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"716\" src=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/blister-packs-of-tablets-1024x716.jpg\" alt=\"Des plaquettes de comprim&#xE9;s\" class=\"wp-image-694\" srcset=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/blister-packs-of-tablets-1024x716.jpg 1024w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/blister-packs-of-tablets-300x210.jpg 300w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/blister-packs-of-tablets-768x537.jpg 768w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/blister-packs-of-tablets-1536x1074.jpg 1536w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-treat-lead__body is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Commencer avant que les d\u00e9g\u00e2ts ne soient faits<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Un nerf perdu ne revient pas. Les l\u00e9sions caus\u00e9es par cette maladie sont g\u00e9n\u00e9ralement irr\u00e9versibles, et aucun traitement ne les fait r\u00e9gresser. Ce que fait le traitement, c\u2019est emp\u00eacher la maladie d\u2019aller plus loin.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Ce seul fait explique tout le reste de cette page\u00a0: pourquoi le d\u00e9lai de diagnostic compte, pourquoi les porteurs sont suivis chaque ann\u00e9e, et pourquoi les familles sont test\u00e9es.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-grid is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><path d=\"M12 22s8-4 8-10V5l-8-3-8 3v7c0 6 8 10 8 10z\"><\/path><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Stabilisateurs de la TTR<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Un stabilisateur, pris chaque jour par voie orale. Il existe deux dosages\u00a0: un plus faible pour la polyneuropathie, un plus \u00e9lev\u00e9 lorsque le c\u0153ur est en cause. Il ralentit la maladie.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><path d=\"M2 15c6.667-6 13.333 0 20-6\"><\/path><path d=\"M2 9c6.667 6 13.333 0 20 6\"><\/path><line x1=\"7\" y1=\"6\" x2=\"9\" y2=\"8\"><\/line><line x1=\"15\" y1=\"16\" x2=\"17\" y2=\"18\"><\/line><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Silenceurs de g\u00e8ne (perfusion)<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Administr\u00e9 en perfusion toutes les trois semaines. Des douleurs abdominales passag\u00e8res et un gonflement des membres sont possibles. Aucune surveillance sanguine syst\u00e9matique n\u2019est n\u00e9cessaire.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><path d=\"M10.5 20.5 3.5 13.5a5 5 0 0 1 7-7l7 7a5 5 0 0 1-7 7Z\"><\/path><path d=\"m8.5 8.5 7 7\"><\/path><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Th\u00e9rapie antisens<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Une injection sous la peau une fois par semaine. Elle demande une surveillance plus \u00e9troite\u00a0: num\u00e9ration des plaquettes tous les quinze jours, et contr\u00f4les r\u00e9guliers de la fonction r\u00e9nale.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><circle cx=\"12\" cy=\"12\" r=\"9\"><\/circle><polyline points=\"12 7 12 12 15.5 14\"><\/polyline><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Silenceurs de g\u00e8ne (injection)<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Inject\u00e9 sous la peau une fois tous les trois mois, \u00e0 l\u2019h\u00f4pital ou \u00e0 domicile. Sans pr\u00e9m\u00e9dication, sans analyses de sang syst\u00e9matiques.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><path d=\"M4 3v10a4 4 0 0 0 8 0V7a3 3 0 0 1 6 0v8\"><\/path><circle cx=\"18\" cy=\"18\" r=\"3\"><\/circle><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Greffe de foie<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Longtemps le seul moyen d\u2019arr\u00eater la production de la prot\u00e9ine mut\u00e9e. Maintenant que des m\u00e9dicaments ont pris le relais, elle n\u2019est indiqu\u00e9e qu\u2019\u00e0 titre exceptionnel.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-treating-card is-layout-flow wp-block-group-is-layout-flow\">\n<span class=\"aa-icon-tile\"><svg aria-hidden=\"true\" focusable=\"false\" width=\"24\" height=\"24\" viewbox=\"0 0 24 24\" fill=\"none\" stroke=\"currentColor\" stroke-width=\"1.75\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><path d=\"M19 14c1.49-1.46 3-3.21 3-5.5A5.5 5.5 0 0 0 16.5 3c-1.76 0-3 .5-4.5 2-1.5-1.5-2.74-2-4.5-2A5.5 5.5 0 0 0 2 8.5c0 2.29 1.51 4.04 3 5.5l7 7Z\"><\/path><\/svg><\/span>\n\n\n\n<h3 class=\"wp-block-heading\">Vivre avec les sympt\u00f4mes<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Des antalgiques sp\u00e9cifiques et des patchs m\u00e9dicamenteux contre les douleurs neuropathiques. Des soins attentifs des pieds, car une plaie qu\u2019on ne sent pas n\u2019est pas soign\u00e9e. Un pacemaker pour les troubles de la conduction. Une vitrectomie pour les d\u00e9p\u00f4ts du vitr\u00e9 qui menacent la vue, avec une bonne r\u00e9cup\u00e9ration visuelle. Des collyres, le laser ou la chirurgie pour le glaucome, et des larmes artificielles contre la s\u00e9cheresse.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-support is-layout-flow wp-block-group-is-layout-flow\">\n<h4 class=\"wp-block-heading\">Si le c\u0153ur est touch\u00e9<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">R\u00e9duire le sel, associ\u00e9 \u00e0 des diur\u00e9tiques, soulage l\u2019essoufflement et les gonflements. Lorsque le rythme est irr\u00e9gulier, un traitement anticoagulant \u00e9vite la formation de caillots dans le c\u0153ur.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Comme dans l\u2019amylose ATTRwt, certains m\u00e9dicaments prescrits couramment dans l\u2019insuffisance cardiaque ordinaire, dont les b\u00eatabloquants et les vasodilatateurs, peuvent aggraver la situation et sont \u00e9vit\u00e9s. Cette d\u00e9cision revient \u00e0 votre cardiologue. N\u2019arr\u00eatez jamais un traitement de vous-m\u00eame.<\/p>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"family\" class=\"wp-block-group aa-section aa-section--cream aa-h-family is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-centered-head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">06<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">G\u00e9n\u00e9tique et famille<\/h2>\n\n\n\n<div class=\"aa-divider-dot\"><\/div>\n\n\n\n<p class=\"wp-block-paragraph\">C\u2019est ce qui distingue l\u2019ATTRv de toutes les autres formes d\u2019amylose\u00a0: elle ne concerne pas une personne \u00e0 la fois.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-treat-lead is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-treat-lead__photo is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"683\" src=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-extended-family-holding-hands-on-a-beach-at-sunset-1024x683.jpg\" alt=\"Une famille &#xE9;largie se tenant la main sur une plage au coucher du soleil\" class=\"wp-image-695\" srcset=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-extended-family-holding-hands-on-a-beach-at-sunset-1024x683.jpg 1024w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-extended-family-holding-hands-on-a-beach-at-sunset-300x200.jpg 300w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-extended-family-holding-hands-on-a-beach-at-sunset-768x512.jpg 768w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/an-extended-family-holding-hands-on-a-beach-at-sunset-1536x1024.jpg 1536w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-treat-lead__body is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\">Un diagnostic qui concerne plus d\u2019une personne<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">L\u2019ATTRv se transmet sur le mode autosomique dominant\u00a0: le variant vient d\u2019un des parents, et chaque enfant d\u2019un porteur a une chance sur deux d\u2019en h\u00e9riter \u00e0 son tour.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">\u00catre porteur du variant ne signifie pas que la maladie se d\u00e9clarera \u00e0 un \u00e2ge pr\u00e9cis\u00a0: deux personnes porteuses de la m\u00eame mutation peuvent tomber malades \u00e0 plusieurs d\u00e9cennies d\u2019\u00e9cart, et certaines ne le seront jamais. Cela signifie en revanche \u00eatre suivi.<\/p>\n<\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-support is-layout-flow wp-block-group-is-layout-flow\">\n<h4 class=\"wp-block-heading\">Le test pr\u00e9symptomatique<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Une fois une mutation identifi\u00e9e dans une famille, un test g\u00e9n\u00e9tique peut \u00eatre propos\u00e9 aux apparent\u00e9s. Ceux qui sont porteurs du variant mais n\u2019ont aucun sympt\u00f4me sont suivis une fois par an, afin de rep\u00e9rer les premiers signes d\u00e8s leur apparition plut\u00f4t que des ann\u00e9es plus tard.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">La raison est simple\u00a0: une fois faites, les l\u00e9sions sont g\u00e9n\u00e9ralement irr\u00e9versibles. Se faire tester ou non reste une d\u00e9cision personnelle, que personne ne devrait avoir \u00e0 prendre seul\u00a0: votre centre est l\u00e0 pour vous aider \u00e0 y r\u00e9fl\u00e9chir.<\/p>\n<\/div>\n<\/div>\n<\/section>\n\n<section id=\"follow-up\" class=\"wp-block-group aa-section aa-section--forest aa-tp-outlook is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-tp-outlook__photo is-layout-flow wp-block-group-is-layout-flow\">\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"1024\" height=\"1024\" src=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-1024x1024.jpg\" alt=\"Des cellules sous un microscope &#xE0; fluorescence\" class=\"wp-image-696\" srcset=\"https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-1024x1024.jpg 1024w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-300x300.jpg 300w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-150x150.jpg 150w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-768x768.jpg 768w, https:\/\/www.amyloidosisalliance.org\/wp-content\/uploads\/2026\/09\/cells-under-a-fluorescence-microscope-1536x1536.jpg 1536w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-outlook__body is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">07<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Suivi et pronostic<\/h2>\n\n\n\n<h4 class=\"wp-block-heading\">Un rythme r\u00e9gulier<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">Une consultation de neurologie tous les six \u00e0 douze mois. Un bilan cardiaque chaque ann\u00e9e\u00a0: examen, ECG, \u00e9chocardiographie, BNP et troponine, avec un Holter si n\u00e9cessaire. Si un pacemaker a \u00e9t\u00e9 pos\u00e9, il est contr\u00f4l\u00e9 tous les six mois. La gastro-ent\u00e9rologie et l\u2019urologie interviennent selon les besoins.<\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Bien plus que des m\u00e9decins<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">La prise en charge s\u2019organise autour d\u2019un centre pluridisciplinaire. Psychologues, assistants sociaux, ergoth\u00e9rapeutes et kin\u00e9sith\u00e9rapeutes en font partie, car une maladie qui atteint les nerfs, l\u2019intestin, les yeux et le c\u0153ur atteint aussi une vie.<\/p>\n\n\n\n<p class=\"aa-tp-outlook__closing wp-block-paragraph\">Sans traitement, la maladie \u00e9volue inexorablement vers un handicap croissant. Trait\u00e9e t\u00f4t, son \u00e9volution change. C\u2019est tout l\u2019argument en faveur du d\u00e9pistage, et du d\u00e9pistage des familles.<\/p>\n<\/div>\n<\/div>\n<\/section>\n\n<section class=\"wp-block-group aa-section aa-section--white aa-tp-faq is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-centered-head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">FAQ<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Questions fr\u00e9quentes<\/h2>\n\n\n\n<div class=\"aa-divider-dot\"><\/div>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-acc is-layout-flow wp-block-group-is-layout-flow\">\n<details class=\"wp-block-details is-layout-flow wp-block-details-is-layout-flow\"><summary>Mes enfants en h\u00e9riteront-ils\u202f?<\/summary>\n<p class=\"wp-block-paragraph\">L\u2019ATTRv se transmet sur le mode autosomique dominant\u00a0: chaque enfant d\u2019un porteur a donc une chance sur deux d\u2019h\u00e9riter du variant. Un test g\u00e9n\u00e9tique peut r\u00e9pondre directement \u00e0 la question. Faire ce test, et quand, est une d\u00e9cision \u00e0 laquelle votre centre peut vous aider \u00e0 r\u00e9fl\u00e9chir.<\/p>\n<\/details>\n\n\n\n<details class=\"wp-block-details is-layout-flow wp-block-details-is-layout-flow\"><summary>Si je suis porteur du variant, vais-je tomber malade\u202f?<\/summary>\n<p class=\"wp-block-paragraph\">Pas forc\u00e9ment, et pas \u00e0 un \u00e2ge pr\u00e9visible. Les sympt\u00f4mes peuvent appara\u00eetre \u00e0 tout moment \u00e0 partir du d\u00e9but de l\u2019\u00e2ge adulte, et l\u2019\u00e2ge de d\u00e9but varie selon le variant et l\u2019origine familiale\u00a0: deux personnes porteuses de la m\u00eame mutation peuvent tomber malades \u00e0 plusieurs d\u00e9cennies d\u2019\u00e9cart. Les porteurs sans sympt\u00f4mes sont suivis une fois par an, afin de rep\u00e9rer la maladie d\u00e8s son tout d\u00e9but.<\/p>\n<\/details>\n\n\n\n<details class=\"wp-block-details is-layout-flow wp-block-details-is-layout-flow\"><summary>L\u2019ATTRv est-elle la m\u00eame maladie que l\u2019ATTRwt\u202f?<\/summary>\n<p class=\"wp-block-paragraph\">La m\u00eame prot\u00e9ine se replie mal dans les deux cas, mais pour des raisons diff\u00e9rentes. Dans l\u2019amylose ATTRwt, le g\u00e8ne est normal et seul l\u2019\u00e2ge d\u00e9stabilise la prot\u00e9ine. Dans l\u2019ATTRv, c\u2019est une mutation h\u00e9r\u00e9ditaire. Elles peuvent se ressembler dans le c\u0153ur\u00a0: c\u2019est pr\u00e9cis\u00e9ment pourquoi un test g\u00e9n\u00e9tique fait partie du diagnostic, et pourquoi la r\u00e9ponse compte pour vos proches, et pas seulement pour vous.<\/p>\n<\/details>\n\n\n\n<details class=\"wp-block-details is-layout-flow wp-block-details-is-layout-flow\"><summary>Les l\u00e9sions nerveuses peuvent-elles r\u00e9gresser\u202f?<\/summary>\n<p class=\"wp-block-paragraph\">En g\u00e9n\u00e9ral, non. Les manifestations de la maladie sont le plus souvent irr\u00e9versibles, et les traitements actuels la ralentissent plut\u00f4t qu\u2019ils ne la r\u00e9parent. C\u2019est pourquoi on insiste autant sur le diagnostic pr\u00e9coce, le suivi annuel des porteurs et le d\u00e9pistage des familles.<\/p>\n<\/details>\n\n\n\n<details class=\"wp-block-details is-layout-flow wp-block-details-is-layout-flow\"><summary>Quelle est la diff\u00e9rence entre l\u2019ATTRv-PN et l\u2019ATTRv-CM\u202f?<\/summary>\n<p class=\"wp-block-paragraph\">L\u2019organe dans lequel la substance amylo\u00efde se d\u00e9pose principalement. Dans l\u2019ATTRv-PN, ce sont les nerfs\u202f; dans l\u2019ATTRv-CM, le c\u0153ur. Mais la distinction est plus nette dans les noms qu\u2019en clinique\u00a0: de tr\u00e8s nombreux patients pr\u00e9sentent un chevauchement important des deux, et le c\u0153ur est souvent touch\u00e9 aussi dans la forme polyneuropathique.<\/p>\n<\/details>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-tp-disclaimer is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"wp-block-paragraph\">Cette page est une information g\u00e9n\u00e9rale destin\u00e9e aux patients, aux familles et aux professionnels de sant\u00e9. Elle ne remplace pas un avis m\u00e9dical. La disponibilit\u00e9 des traitements cit\u00e9s ici varie d\u2019un pays \u00e0 l\u2019autre. Parlez toujours de votre situation avec votre \u00e9quipe sp\u00e9cialis\u00e9e.<\/p>\n<\/div>\n<\/div>\n<\/section>\n\n<section class=\"wp-block-group aa-next aa-next--split is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-inner is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-next__head is-layout-flow wp-block-group-is-layout-flow\">\n<p class=\"aa-section-num wp-block-paragraph\">Continuer<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Autres types<\/h2>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-next__grid is-layout-flow wp-block-group-is-layout-flow\">\n<div class=\"wp-block-group aa-next-card is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\"><a href=\"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/amylose-attrwt\/\">ATTRwt<\/a><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">La forme wild-type\u00a0: la m\u00eame prot\u00e9ine, d\u00e9stabilis\u00e9e par l\u2019\u00e2ge plut\u00f4t que par une mutation.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-next-card is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\"><a href=\"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/amylose-al\/\">Amylose AL<\/a><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">La forme syst\u00e9mique la plus fr\u00e9quente, due aux cha\u00eenes l\u00e9g\u00e8res produites dans la moelle osseuse.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-group aa-next-card is-layout-flow wp-block-group-is-layout-flow\">\n<h3 class=\"wp-block-heading\"><a href=\"https:\/\/www.amyloidosisalliance.org\/fr\/types-damylose\/amylose-aa\/\">Amylose AA<\/a><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">Une complication d\u2019une inflammation de longue dur\u00e9e, qui touche avant tout les reins.<\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/section>\n","protected":false},"excerpt":{"rendered":"<p>Accueil\u203aAmylose\u203aTypes d\u2019amylose\u203aAmylose ATTR\u203aATTRv Amylose ATTR Amylose ATTRv L\u2019amylose ATTR h\u00e9r\u00e9ditaire, aussi appel\u00e9e amylose ATTR variante (ATTRv), est due \u00e0 un variant pathog\u00e8ne du g\u00e8ne TTR, h\u00e9rit\u00e9 d\u2019un parent. C\u2019est la m\u00eame prot\u00e9ine que dans l\u2019amylose ATTRwt, mais d\u00e9stabilis\u00e9e d\u2019embl\u00e9e, et non par le seul effet de l\u2019\u00e2ge. Elle peut toucher les nerfs, le c\u0153ur, ou [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":1427,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-1415","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.6 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Amylose ATTRv\u00a0: la forme h\u00e9r\u00e9ditaire de l\u2019amylose ATTR<\/title>\n<meta name=\"description\" content=\"L\u2019amylose ATTRv est due \u00e0 un variant h\u00e9r\u00e9ditaire du g\u00e8ne TTR. 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