{"id":153,"date":"2026-02-08T13:21:19","date_gmt":"2026-02-08T12:21:19","guid":{"rendered":"https:\/\/erlaxatu.fr\/?page_id=153"},"modified":"2026-03-15T21:16:05","modified_gmt":"2026-03-15T20:16:05","slug":"__trashed-4","status":"publish","type":"page","link":"https:\/\/erlaxatu.fr\/index.php\/__trashed-4\/","title":{"rendered":"Vascularites syst\u00e9miques"},"content":{"rendered":"

Vascularites syst\u00e9miques<\/strong><\/p>\n

I. Pour comprendre<\/strong><\/p>\n

A. D\u00e9finition<\/strong><\/p>\n

Une vascularite correspond \u00e0 une atteinte inflammatoire de la paroi des vaisseaux (art\u00e8res, capillaires ou veines), responsable de manifestations cliniques d\u00e9pendant du type<\/strong> et du nombre<\/strong> de vaisseaux atteints. <\/p>\n

B. Principes de classification<\/strong><\/p>\n

Plusieurs crit\u00e8res existent (calibre, syst\u00e9mique vs localis\u00e9e, association aux ANCA, histologie, primitive vs secondaire). La classification de r\u00e9f\u00e9rence actuelle est la nomenclature de Chapel Hill (2012)<\/strong>, organis\u00e9e surtout selon la taille des vaisseaux atteints.<\/p>\n

C. Vascularites secondaires<\/strong><\/p>\n

\u00c0 \u00e9voquer devant : cause infectieuse, toxique\/m\u00e9dicamenteuse, maladie syst\u00e9mique associ\u00e9e (PR, LES, autres connectivites) ou n\u00e9oplasie. <\/p>\n

II. Quand \u00e9voquer une vascularite ?<\/strong><\/p>\n

A. Signes d\u2019alerte g\u00e9n\u00e9raux<\/strong><\/p>\n

Les pr\u00e9sentations sont polymorphes, mais signes g\u00e9n\u00e9raux<\/strong>, atteintes musculosquelettiques<\/strong> et l\u00e9sions cutan\u00e9es<\/strong> sont fr\u00e9quents dans de nombreuses vascularites. <\/p>\n

led vascu ppr<\/p>\n

B. Focus cutan\u00e9 : le purpura vasculaire<\/strong><\/p>\n

Les vascularites des petits et moyens vaisseaux s\u2019accompagnent volontiers d\u2019un purpura vasculaire.<\/p>\n

Deux m\u00e9canismes \u00e0 distinguer (tr\u00e8s utile en clinique) :<\/strong><\/p>\n

    \n
  1. Inflammation pari\u00e9tale<\/strong> \u2192 purpura infiltr\u00e9\/palpable<\/strong>, souvent polymorphe<\/strong>.<\/li>\n
  2. Processus endoluminal (thrombose\/emboles)<\/strong> \u2192 l\u00e9sions plut\u00f4t monomorphes<\/strong> et n\u00e9crotiques<\/strong> : livedo en faveur d\u2019une thrombose, n\u00e9croses distales pour emboles.<\/li>\n<\/ol>\n

    Le purpura peut aussi \u00e9voluer de fa\u00e7on progressive (pigment\u00e9 des MI ou ecchymotique par fragilit\u00e9 pari\u00e9tale).<\/p>\n

    III. Outils diagnostiques<\/strong><\/p>\n

    A. Bilan biologique de premi\u00e8re intention (hors ANCA)<\/strong><\/p>\n

    \u00c0 adapter au contexte, mais classiquement : syndrome inflammatoire (VS\/CRP\/\u00e9lectrophor\u00e8se), NFS (hyper\u00e9osinophilie), bilan h\u00e9patique, bilan r\u00e9nal + BU + prot\u00e9inurie (id\u00e9alement rapport prot\u00e9ine\/cr\u00e9atinine<\/strong>), recherche h\u00e9maturie glom\u00e9rulaire, s\u00e9rologies virales (VHB, VHC, VIH), recherche d\u2019infections bact\u00e9riennes d\u00e9clenchantes, ANA typ\u00e9s, FR\/anti-CCP, cryoglobulin\u00e9mie et typage.<\/p>\n

    B. Int\u00e9r\u00eat des ANCA<\/strong><\/p>\n

    Les ANCA ont une forte sp\u00e9cificit\u00e9 pour le groupe des vascularites n\u00e9crosantes syst\u00e9miques. D\u00e9pistage initial par immunofluorescence (c-ANCA vs p-ANCA), puis identification antig\u00e9nique par ELISA (PR3, MPO). Le titre<\/strong> n\u2019est pas formellement corr\u00e9l\u00e9 \u00e0 l\u2019activit\u00e9 clinique.<\/p>\n

    C. Recherche de complications visc\u00e9rales (bilan d\u2019extension)<\/strong><\/p>\n

    Le bilan d\u00e9pend du type de vascularite suspect\u00e9e :<\/p>\n

      \n
    • Gros vaisseaux<\/strong> : \u00e9cho Doppler temporale (halo), TEP-FDG pour atteinte aortique\/branches (\u00e0 r\u00e9server aux cas difficiles, attention sp\u00e9cificit\u00e9 chez le sujet \u00e2g\u00e9\/ath\u00e9rome).<\/li>\n
    • Vascularites \u00e0 ANCA et autres<\/strong> : bilan h\u00e9patique, r\u00e9nal (prot\u00e9inurie\/h\u00e9maturie\/rapport prot\/cr\u00e9at), pulmonaire (Rx, scanner, EFR avec DLCO, fibroscopie + LBA si suspicion h\u00e9morragie alv\u00e9olaire avec score de Golde), ORL (examen + scanner), cardio (ECG syst\u00e9matique, ETT \u00b1 troponine\/NT-proBNP\/IRM si besoin), neuro (EMG si atteinte p\u00e9riph\u00e9rique, IRM si atteinte centrale).<\/li>\n<\/ul>\n

      D. Confirmation histologique<\/strong><\/p>\n

      Souvent n\u00e9cessaire (sauf tableau typique) :<\/p>\n

        \n
      • biopsie cutan\u00e9e (la plus simple),<\/li>\n
      • biopsie neuromusculaire si neuropathie,<\/li>\n
      • biopsie d\u2019art\u00e8re temporale (si suspicion ACG),<\/li>\n
      • ponction-biopsie r\u00e9nale selon crit\u00e8res (prot\u00e9inurie + h\u00e9maturie glom\u00e9rulaire, d\u00e9gradation inexpliqu\u00e9e, etc.).<\/li>\n<\/ul>\n

        IV. Moyens th\u00e9rapeutiques (principes)<\/strong><\/p>\n

        A. Objectifs et strat\u00e9gie<\/strong><\/p>\n

        Objectifs : r\u00e9mission\/gu\u00e9rison, limiter rechutes, s\u00e9quelles, iatrog\u00e9nie, am\u00e9liorer qualit\u00e9 de vie. \u00c9tapes : \u00e9viction toxique\/m\u00e9dicament, traiter infection\/cancer, discuter antiagr\u00e9gant\/anticoagulant si facteurs associ\u00e9s, d\u00e9buter rapidement corticoth\u00e9rapie, discuter immunosuppresseur d\u2019induction puis un traitement d\u2019entretien.<\/p>\n

        B. Induction \/ entretien (exemples)<\/strong><\/p>\n

          \n
        • Formes s\u00e9v\u00e8res : cyclophosphamide ou rituximab + cortico\u00efdes, puis relais par immunosuppresseur d\u2019entretien (m\u00e9thotrexate, azathioprine\u2026).<\/li>\n
        • Rituximab<\/strong> : valid\u00e9 induction et entretien des vascularites \u00e0 ANCA (alternative au cyclophosphamide, sans supprimer la n\u00e9cessit\u00e9 d\u2019entretien). <\/li>\n<\/ul>\n

          led vascu ppr<\/p>\n

            \n
          • Plasmaph\u00e9r\u00e8ses : discut\u00e9es en insuffisance r\u00e9nale s\u00e9v\u00e8re et peut-\u00eatre h\u00e9morragie alv\u00e9olaire ; PAN li\u00e9e au VHB : courte corticoth\u00e9rapie + \u00e9changes plasmatiques + antiviral.<\/li>\n<\/ul>\n

            C. Points \u201c\u00e0 savoir dire\u201d \u00e0 l\u2019examen<\/strong><\/p>\n

              \n
            • Urgence th\u00e9rapeutique possible (pronostic vital). <\/li>\n
            • Toujours rechercher une atteinte visc\u00e9rale, notamment r\u00e9nale.<\/li>\n
            • Diagnostic clinicobiologique, biopsie + ANCA utiles.<\/li>\n<\/ul>\n","protected":false},"excerpt":{"rendered":"

              Vascularites syst\u00e9miques I. Pour comprendre A. D\u00e9finition Une vascularite correspond \u00e0 une atteinte inflammatoire de la paroi des vaisseaux (art\u00e8res, […]<\/p>\n","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"site-sidebar-layout":"default","site-content-layout":"","ast-site-content-layout":"default","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","ast-disable-related-posts":"","theme-transparent-header-meta":"","adv-header-id-meta":"","stick-header-meta":"","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-4)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-4)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-4)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"footnotes":""},"class_list":["post-153","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/pages\/153","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/comments?post=153"}],"version-history":[{"count":1,"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/pages\/153\/revisions"}],"predecessor-version":[{"id":159,"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/pages\/153\/revisions\/159"}],"wp:attachment":[{"href":"https:\/\/erlaxatu.fr\/index.php\/wp-json\/wp\/v2\/media?parent=153"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}