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Artérite ya ba cellules géantes (Artérite temporelle): Claudication ya mbanga, kozala tendre ya loposo ya mutu, diplopie, mpe ba stéroïdes ya dose makasi mbala moko liboso ya biopsie mpo na kobikisa liso ya mibale

Mituya ya mbalakaka ekeseni na mikili. Lokasa oyo esaleli 911, nimero ya États-Unis mpe Canada. Bikólo mosusu mingi esalelaka 112, 999, to motángo mosusu. Luka nimero ya mboka na yo.

Na mokóló nyonso oyo azali na mbula 50 to koleka, ebandeli ya mbalakaka ya mpasi ya motó ya sika ya tempelo, kolɛmba ya loposo ya motó ntango ozali kokata nsuki, mpasi ya kolɛmba na misisa ya mbanga ntango azali kotafuna bilei (claudication ya mbanga), kokita ya komona mpo na mwa ntango moke, komona mbala mibale (diplopie), to kokufa miso na mbalakaka na liso moko ezali likambo ya mbalakaka ya monganga oyo eutaka na artérites ya baselile minene (GCA), eyebani mpe na kombo ya artérite temporelle to maladi ya Horton. Benga nimero ya urgence ya esika ofandi (911 na Etats-Unis mpe Canada, 112 na Union européenne mobimba, 999 na Royaume-Uni) to tuna ya biso banimero ya mbalakaka mbala moko mpe kende mbala moko na departema ya urgence ya lopitalo mpo na stat haute dose intraveineux corticostéroïdes. Ntango ozali kobongisa transport, kokesenisa kobungisa vision artérielle na thromboembolique non artérienne occlusion ya artérie rétine centrale (CRAO) ya miso, zongelá makambo na biso ya monene kobungisa komona na mbalakaka mpe guide ya détachement ya rétine, mpe tala soki bilembo ya cerveau ezali komonana mbala moko avc.

Artérite cellulaire géante ezali vasculite granulomatose systémique, médiée par cellules T oyo e attaquer ba artéries ya taille moyenne mpe ya minene oyo ezali na lamina élastique interne, na prédilection sans relâche mpo na ba branches extracrâniennes ya ba artéries carotides externe mpe interne, surtout artérie temporelle superficielle, artérie maxillaire, artérie ophtalmique, mpe ba artéries ciliaires ya sima ya mikuse. Ntango hyperplasie intime inflammatoire evimbi bifelo ya ba artéries ciliaires postérieures mikuse tii ntango ba lumens na yango ekokangama, motó ya misisa ya miso ezwamaka na infarctus catastrophique (neuropathie optique ischémique antérieure artérienne [A-AION]), oyo epesaka aveugle ya mozindo, oyo ekoki kobongwana te (mbala mingi tii na kososola pole te). Soki komona ebungaki na liso ya liboso, ezongaka mbala mingi te ata na nkisi ya makasi. Nzokande, soki basalisi yango mbala moko te na dose monene ya corticostéroïde, bato 30 kino 50 likoló na monkama bakozwa miso ekufá miso mpo na libela, mobimba na liso na bango ya mibale (moninga na bango) na boumeli ya ngonga 24 tii mikolo 7. Mabongisi ya mikili mingi oyo euti na Collège américain d’héumatologie (ACR), EULAR, mpe Société britannique pour la réumatologie (BSR) esɛngi ete minganga ya nokinoki bápesa na misisa méthylprednisolone (500 kino 1.000 milligrammes mokolo na mokolo) to prednisone orale ya dose makasi mbala moko na suspect clinique, kozanga kozela bangonga to mikolo mpo na ba résultats ya laboratoire ya taux ya sédimentation ya ba érythrocytes (ESR), ultrasons ya artérie temporelle, to biopsie ya artérie temporelle.

Sala boye sikoyo

  1. Kende mbala moko na departema ya urgence ya lopitalo (clinique ya soins ya urgence te, bureau ya mino, to clinique ya optométrie routine) soki mokóló ya mibu 50 to koleka azwi mbongwana ya komona TO bilembo ya artérite crânienne classique. Salisa bomoni ya brume transitoire (amaurose fugax), ndambo ya likolo to ya nse ya komona oyo ezangi (défaut altitudinal), vision double (diplopie), to mpasi ya motó ya sika na claudication ya mbanga lokola likama ya mbala moko mpo na komona na ngambo mibale.
  2. Yebisa infirmier ya triage ya urgence mpe monganga na fraze na yo ya liboso mpenza: "Tozali kokanisa ete Artérite Cellule géante ezali na likama ya komona mpe tosengeli na ba stéroïdes intraveineux ya dose makasi ya mbala moko mpo na kobikisa liso ya mibale." Kozela te na ndenge ya pasif na salle de zela ya lopitalo ntango ozali kosimba liso oyo ezali komonana te. Ngonga nyonso ya retard liboso ya dose ya liboso ya méthylprednisolone intraveineuse (500 kino 1.000 mg IV) ematisaka likama ete cascade inflammatoire ekokanga ba artéries ciliaires ya sima ya moninga ya liso.
  3. Kotika ata moke te monganga azongisa sima kobanda ba corticostéroïdes tango azali kozela biopsie ya artérie temporelle to ultrasons ya ambulatoire. Libunga ya monganga oyo emonanaka mingi mpe ya kobebisa ezali kopekisa prednisone to méthylprednisolone mpo na kobanga ete ba stéroïdes eko "bebisa" résultat ya biopsie ya artérie temporelle. Inflammation granulomatose pathologique mpe ba cellules géantes etikalaka komonana polele na mur ya artérie temporelle pendant au moins 10 à 14 jours (mpe mbala mingi jusqu’à 4 semaines) sima ya kobanda ba corticostéroïdes ya dose makasi. Ba stéroïdes nde eyaka liboso; biopsie ezali na esika ya mibale.
  4. Yeba "claudication ya mbanga" mpe "diplopie" lokola bilembo ya likebisi ya liboso ya kokufa miso oyo ezali koya. Soki misisa ya mbanga na yo ezali kosala mpasi, kolɛmba, to kolɛmba nsima ya miniti 1 kino 2 ya kotafuna misuni, mampa ya kopɔla, to bilei ya makasi mpe koyoka malamu ntango otiki kotafuna, yango ezali T trouble ya articulation temporomandibulaire (TMJ) to mino ya mabe. Ezali claudication ischémique ya ba muscles masseter na temporalis oyo esalemi na réduction inflammatoire ya ba artéries maxillaire na elongi, mpe ememaka corrélation statistique ya likolo na aveugle oyo ezali koya na GCA. Ndenge moko mpe, komona mbala mibale mpo na mwa ntango moke liboso ya kokufa miso mpo na libela na bangonga to mikolo epai ya bato ya maladi tii 15 %.
  5. Lalisa mobeli na plat (supine) soki perte ya vision aiguë to dimming transitoire ebandi déjà. Kolala plat e maximiser pression ya perfusion artérielle systémique na nzela ya ba artéries ophtalmiques mpe ciliaires postérieures rétroites oyo ezali koleisa mutu ya nerf optique tango bazali kobongisa méthylprednisolone intraveineuse.
  6. Tala bilembo ya AVC FAST mpe bilembo ya vertebrobasilaires. Lokola artérite cellulaire géante epelisaka mpe artéries vertébrales mpe artéries carotides internes na 3 kino 7% ya ba maladi, tala soki kizunguzungu ezali, vertigo, marche insteady, maloba ya mabe, to bolembu ya ngambo moko, mpe koyebisa yango mbala moko na équipe ya urgence.

Mpo na nini kozela ekzamɛ ya makila to ya biopsie ekangaka liso ya mibale

Na artérite ya ba cellules géantes oyo ezwami na traitement te na perte ya vision unilatérale, un tiers to ndambo ya ba malades bakendaka libela aveugle na liso na bango ya malamu oyo etikali na 1 à 7 jour, tango mosusu na moins de 24 heures. En plus, jusqu’à 4 à 10 pour cent ya ba malades ya GCA oyo ba prouvé na biopsie bazali na ESR normal to CRP normal, elingi koloba test ya makila normal elongolaka jamais GCA tango ba symptômes cliniques ezali compulsif. Minganga ya mbalakaka basengeli kobimisa makila mpo na ESR, CRP, mpe ba plaquettes mpe kobanda 500 kino 1.000 mg ya méthylprednisolone na misisa mbala moko na esika yango, na nsima kosala programme ya ultrasons ya artérie temporelle mpe biopsie ya artérie temporelle na mikolo 7 kino 14 oyo ekolanda.

Nzete ya mikano ya clinique mpo na oyo ba suspecter Artérite Cellule géante na mikóló ya mibu 50 mpe koleka

Salelá algorithme oyo ya triage clinique mpo na ko stratifier ba malades oyo ba suspecter GCA na ba urgences visuelle/ischémiques contre GCA crânienne non ischémique mpe kobanda kobikisa mbala moko na ba stéroïdes.

flowchart TD
    Start["Age ya adulte >= 50 Ans Ko présenter na NIOSO:<br/>1. Motó mpasi oyo ebandi sika (Temple, occipital, to généralisé)<br/>2. Claudication ya mbanga to ya monoko tango ya kotafuna<br/>3. Tendresse ya loposo ya mutu to Artérie temporale nodulaire/sans pulsée<br/>4. Komona na mbalakaka, Amaurose Fugax, Diplopie, to Kokufa miso<br/>5. Fièvre oyo ekoki kolimbolama te, Kokita ya kilo, to Polymyalgie Rheumatica"] --> VisualCheck{"Est-ce que Ezali na bilembo ya Ischémique ya komona to ya motó<br/>Ezali na bilembo ya likebisi?<br/>(Kobungisa komona, Amaurose Fugax, Diplopie,<br/>to Claudication ya mbanga/Monoko)"}

    VisualCheck -->|"OUI: URGENCE OCULAIRE / ISCHÉMIQUE<br/>(Risque Imminent ya likolo ya aveugle bilatérale<br/>to AVC Vertebrobasilaire)"| EmergencyIV["PROTOCOL YA DEPARTEMENT YA URGENCE YA MBONGO:<br/>1. KOZELA TE ba résultats ya ESR/CRP to Biopsie<br/>2. Kopesa Dose ya likolo IV Méthylprednisolone<br/> 500 kino 1.000 mg IV Mokolo na Mokolo na Mikolo 3 Milandani<br/> (To Prednisone 60 kino 80 mg PO Soki IV ezongisami sima)<br/>3. Lalisa Mobɛli Plat (Supine) mpo na ko Maximiser Perfusion<br/>4. Dessin Stat ESR, CRP, CBC (Platelets), Fibrinogène"]

    EmergencyIV --> Maintenance["Nsima ya Mikolo 3 ya ba Stéroïdes ya Pulse IV:<br/>1. Bobongwani na Prédnisone ya monoko 1 mg/kg/mokolo<br/> (mingimingi 60 mg mokolo na mokolo) + Kobakisa Tocilizumab (Blocker IL-6)<br/>2. Kosala Ultrason ya Artérie Temporal (‘Elembo ya Halo’)<br/> mpe Biopsie ya Artérie Temporal Unilatérale/Bilatérale<br/> (> bolai ya 1,5 kino 2,0 cm) Na Mikolo 7 kino 14<br/>3. CTA/MRA Aorta & Branches mpo na GCA ya masuwa ya minene"]

    VisualCheck -->|"NON: Motó mpasi ya motó, Tendresse ya loposo ya motó,<br/>to Symptômes ya PMR KAKA<br/>(Vision 100% Normale, Claudication ya mbanga te)"| NonIschemic["PROTOCOL YA GCA YA MOKOLO MOKO YA URGENT:<br/>1. Benda Stat ESR, CRP, mpe Motango mobimba ya Makila<br/>2. Bandá komɛla Prednisone na monɔkɔ na dose monene mbala moko:<br/> 40 kino 60 mg ya PO Mokolo na mokolo (1 mg/kg/mokolo) YAMBI YA Biopsie<br/>3. Ultrasons ya artérie temporelle ya noki noki<br/> mpe Biopsie ya artérie temporelle na kati ya poso 1 to 2<br/>4. Teya Mobɛli azonga na ER Mbala moko soki<br/> Dimming Visual, Diplopie, to Pasi ya Mbala Esalemi"]

Physiopathologie: Ndenge nini vasculite granulomatose auto-immune ekanga misisa ya miso

Kososola ntina oyo artérite ya baselile minene etalelaka misisa ya sikisiki ya mikóló oyo baleki mbula 50 elimboli ezala kosangisama na yango ya kokamwa ya bilembo (mpasi ya mbanga, kolɛmba ya loposo ya motó, kokangama ya mapeka, mpe kokufa miso na mbalakaka) mpe mpo na nini bankisi oyo bato mingi basalelaka mpo na kosilisa kokangama ya miso ekoki te kozongisa nsima infarctus ya misisa ya miso.

1. Mpo na nini Artérite Cellule géante ezwamaka kaka na mikóló ya mbula 50 mpe koleka

Artérite ya ba cellules géantes presque ezwamaka ata moke te na bato oyo bazali na nse ya mbula 50, mpe bokono na yango emati makasi na mbula zomi moko na moko oyo eleki mbula 50, ekómaka na nsɔngɛ kati na mbula 70 mpe 80 ans (na bopanzani mingi na ba populations ya bankɔkɔ ya Europe du Nord mpe ya Scandinavie, mpe ratio ya 2:1 kino 3:1 ya basi na mibali). - Senescence ya Lamine élastique na ba cellules dendritiques adventitiales: ba artéries moyennes na ya minene ezali na lamina élastique interne (ring ya ba fibres élastiques oyo ekabolaka intima ya kati na ba milieu musculaire) mpe e nourrir na ba veineux microscopiques ya makila na mur na yango ya libanda oyo babengaka vasa vasorum. Na vieillissement eleki âge 50, ba changements structurels liés na âge na ba fibres ya élastine mpe na sénescence immunitaire e activaka ba récepteurs spécialisés oyo ezali lokola toll na ba cellules dendritiques adventitiales autochtones oyo efandaka na mur ya libanda ya ba artéries crâniennes mpe na aorta thoracique. - Macrophage T-Cell Cascade Granulomatose: Ba cellules dendritiques activées e recruter CD4+ T lymphocytes na nzela ya vasa vasorum na nzela ya ba voies inflammatoires mibale ekeseni: 1. Nzela ya Interleukin-6 (IL-6) mpe IL-17 (Th17), oyo etambwisaka ba réactifs systémiques ya phase aiguë hépatique (ESR, CRP, fibrinogène, mpe thrombocytose réactive oyo ezali komata na lola) mpe epesaka fièvre, suki ya butu, kokita ya kilo, mpe polymyalgie rheumatique (PMR); mpe 2. Interféron-gamma (IFN-\(\gamma\), Th1) mpe nzela ya macrophage, oyo e déclenchaka ba macrophages e fusionner na ba cellules géantes multinucléées oyo ebimisaka ba métalloprotéinases matrice (ebebisaka lamine élastique interne) mpe facteur de croissance dérivé na ba plaquettes (PDGF) mpe facteur de croissance endothélial vasculaire (VEGF). - Occlusion Luminal par Hyperplasie Intimal (Clot ya makila ya pete te): Na se ya stimulation ya PDGF, ba cellules ya muscle lisse vasculaire ekendeke na kati mpe e proliférer noki, epesaka hyperplasie intimale concentrique massive. Revêtement ya kati ya artérie evimbaka na kati tii tango canal central (lumen) ekofinama na se tii na libulu ya pin to 100 pour cent ekangama.

+-------------------------------------------------------------------------+
|     VASCULAR TARGETS OF GIANT CELL ARTERITIS (CAROTID BRANCHES)         |
+-------------------------------------------------------------------------+
|  EXTERNAL CAROTID ARTERY BRANCHES:                                      |
|  * Superficial Temporal Artery ---> Throbbing temple headache, tender   |
|    scalp when combing hair, thick cord-like pulseless artery, scalp     |
|    necrosis.                                                            |
|  * Maxillary & Facial Arteries ---> JAW CLAUDICATION (ischemic cramping |
|    of masseter/temporalis muscles after 1-2 min of chewing food).       |
|  * Lingual Artery -------------> Tongue claudication, blanching, or     |
|    ischemic tongue necrosis while eating or speaking.                   |
+-------------------------------------------------------------------------+
|  INTERNAL CAROTID ---> OPHTHALMIC ARTERY BRANCHES (BLINDING TARGETS):   |
|  * Short Posterior Ciliary Arteries (80%-85% of GCA Blindness):         |
|    ---> Infarcts the optic nerve head = ARTERITIC ANTERIOR ISCHEMIC     |
|         OPTIC NEUROPATHY (A-AION) with a "chalky-white" swollen disc.   |
|  * Central Retinal Artery (5%-10% of GCA Blindness):                    |
|    ---> Arteritic CRAO (milky-white retina + cherry-red spot).          |
|  * Muscular/Cranial Nerve Vasa Vasorum (10%-15% Pre-Blindness Sign):    |
|    ---> Ischemia of extraocular muscles or CN III, IV, VI = DIPLOPIA.   |
+-------------------------------------------------------------------------+

2. Pourquoi occlusion ya artérie ciliaire postérieure esalaka Infarctus nerveux optique "Blanc-chalque" (A-AION) .

Alors que artérie temporelle superficielle na elongi ezuaka surnom ya maladie, culpable ya solo ya aveugle na kati ya orbite ezali presque toujours occlusion inflammatoire ya ba artéries ciliaires postérieures courtes (SPCAs): - Kati na 10 na 20 ba artéries ciliaires ya sima ya mikuse epanzani na artérie ophtalmique mpe etɔbɔlaka ba scléres na cercle (cercle ya Zinn-Haller) directement zinga zinga ya tête ya nerf optique (disque optique) mpo na ko fournir mutu ya nerf optique prélaminaire mpe laminaire mpe lisusu choriocapillaris choroïdale mobimba. - Na 80 kino 85% ya ba maladi oyo bazali na bobungisi ya komona na GCA, vasculite granulomatose ekangamaka na ba artéries ciliaires ya sima ya mikuse, mpe epesaka Neuropathie optique ischémique antérieure arteritique (A-AION). - Na fundoscopie, mutu ya nerf optique infarcté evimbaka mpe ebalukaka œdème pathognomique "blanc-craie" (pallide), ekeseni mpenza na kovimba ya rose/hypérémique oyo emonanaka na AION bénine non-artéritique to papillédème. - Na 5 kino 10 pour cent mosusu, GCA ekangaka artérie rétinienne centrale (CRAO artéritique), mpe na 5 pour cent ekangaka ba vaisseaux ciliaires postérieurs rétrobulbaires sima ya globe (Neuropathie optique ischémique postérieure [PION], esika mobɛli azali acutement aveugle na pupille ya Marcus Gunn atako disque optique ezali komonana totalement normal na mokolo ya liboso).

Koyeba ba 6 syndromes cliniques cardinaux ya Artérite Cellulaire Giante

Lokola artérite ya baselile minene ekoki komimonisa lokola migraine, mokakatano ya mino/TMJ, maladi ya sinus, arthrite, to maladi mabe ya kobombama, minganga mpe bato ya maladi basengeli koyeba bituluku motoba ya bilembo na yango ya kala:

1. Claudication ya mbanga mpe claudication ya monoko (Bilembo ya sikisiki mingi) .

  • Claudication ya mbanga (Ezali na 40% à 50% ya ba malades ya GCA): Na bokeseni na arthrite ya articulation temporomandibulaire (TMJ), oyo epesaka clic makasi to mpasi ya mbala moko na moment oyo mutu afungoli monoko na ye monene to aswaki na se na eloko ya makasi, claudication ya mbanga ezali ndenge moko na angina ya motema to claudication intermittent ya ba ngɔmbɛ. Ntango mobɛli abandi kotafuna bilei ya makasi (na ndakisa bisteki, ngulu, bagels, to mampa oyo elɛmbisaka nzoto), mwa ndambo ya liboso oyo aswaki yango ezalaka mpenza mpasi te. Nsima ya segɔnde 60 kino 120 ya kotafuna mbala na mbala, misisa ya masseter mpe ya temporalis esilaka oxygène mpo artérie maxillaire oyo evimbaki ekoki te kobakisa koleka ya makila; kolɛmba ya mozindo, ya kolɛmba mpe ya mpasi ekómaka na mipanzi ya mbanga, mpe yango etindaka moto yango atika kotafuna mpe apema. Na boumeli ya miniti 1 to 2 nsima ya kopema mbanga, mpasi yango esilisaka, kaka ezongaka ntango kaka ebandi lisusu kotafuna. Mobɛli oyo azali na GCA mpe claudication ya mbanga azali na likama ya aveugle permanente mbala 3 kino 4 koleka mobɛli ya GCA oyo azali na claudication ya mbanga te.
  • Claudication ya monoko mpe mpasi ya zolo: Ischémie ya ba artéries pharyngées linguales mpe ascendantes epesaka mpasi ya cramping to etumbu na monoko tango ozali koloba to kolia, mpasi na komela (odynophagie), to nsɔngɛ ya monoko ya pâle/blanché oyo ekoki kokende liboso na franque gangrene/nécrose ya monoko soki ba diagnostiquer yango mabe.

2. Motó mpasi oyo ebandi sika, loposo ya motó ezali kolɛmba, mpe misisa ya motema oyo ezali malamu te

  • Motó mpasi oyo ebandi sika (Ezali na 70% kino 85%): Lolenge nyonso ya sika ya mpasi ya motó na mokóló oyo azali na mbula koleka 50 (to mbongwana monene na lolenge ya mpasi ya motó ya moto oyo azali na maladi ya motó na bomoi mobimba) ezali drapeau rouge. Alors que classiquement pasi constant, ennuyeux, to throbbing likolo ya temples moko to nionso mibale, mpasi ya mutu ya GCA ekoki kozala frontal, occipital (na sima ya crâne via inflammation ya artérie occipital), to généralisée na kati ya vertex.
  • Tenderness ya loposo ya mutu (Allodynia): Inflammation ya ba branches superficielles ya artérie temporelle na occipitale oyo e fournir loposo ya loposo ya mutu epesaka tendresse exquise tango ozali ko peigner to ko brosser suki, kosukola suki na douche, kolata ba lunettes to chapeau, to ko lalisa mutu na oreiller na butu. Na ba cas ya makasi oyo esalemi te, ba ulcères croûteuses ya mpasi (nécrose ya mutu) ebimaka likolo ya poso ya mutu ya frontopariétal mpe na ebandeli ekoki kozala na libunga ya ba shingles (herpès zoster).
  • Examen physique ya artérie temporale superficielle: Kosimbasimba na malembe liboso ya litoi (tragus) mpe pembeni ya temple ekoki komona artérie temporal superficielle oyo eyokaka ezali épaisse, nodular ("perles"), makasi lokola eteni ya nsinga to nsinga, tendre mpo na kosimba, érythématose, mpe na pulse oyo elɛmbi to oyo ezali mpenza te.

3. Bilembeteli ya likebisi oyo emonanaka na miso: Amaurose fugax, diplopie, mpe A-AION

  • Bobungisi ya komona na monoculaire transitoire (Amaurosis Fugax na 20% kino 30%): Mikolo to bangonga yambo ete bokufi miso ya libela esala, kino moko na misato ya ba maladi bazwaka ba épisodes ya mokuse oyo eumelaka miniti 1 kino 15 esika wapi komona na liso moko emonanaka lokola brume, esukolami lokola fɔtɔ́ oyo ezali koleka ndelo, to ezipami na elili ya molili, ntango mosusu ebandisami kaka na kotelema uta kiti moko (ischémie ya nerf optique postural mpo tansion à peine Trickles koleka mur ya artérie oyo evimbi).
  • Diplopie transitoire to persistente (Double Vision na 6% à 15%): Tango GCA e inflammer ba petites artéries nutriantes oyo epesaka ba muscles extraoculaires to ba nerfs crâniens ya misato, ya minei, to ya motoba na kati ya orbite, miso mibale ebungisaka alignment, epesaka brusquement horizontal to vertical double vision (oyo elimwaka tango ezipaka moko to mosusu liso). Na mokóló oyo azali na mbula koleka 50 oyo azali na mpasi ya motó to mpasi ya mbanga, diplopie transitoire ezali likambo ya mbalakaka ya ba alarmes mitano liboso ya kokufa miso.
  • Aveugle altitudinal to totale ya libela: Ba malades balamukaka mingi na tongo to bamoni na mbalakaka na moyi que ndambo mobimba ya se to ndambo ya likolo ya vision na liso moko esili (défaut ya champ visuel altitudinal), to ete liso mobimba ekendeki molili tii na kotanga misapi to perception ya lumière te (NLP).

4. Polymyalgie Rheumatica (PMR) mpe bilembo ya B systémiques

  • Polymyalgie Rheumatica (Ezali na 40% kino 50% ya ba maladi ya GCA): Contrairement, pene na 15 kino 20% ya ba maladi oyo bazwami na PMR bazali na GCA ya se to oyo elandi. PMR epesaka mpasi makasi na ngambo mibale mpe kokangama ya ntongo oyo eumelaka koleka miniti 45 kino 60 na kati ya ba ceintures ya mapeka, nkingo, mpe ba ceintures pelvises/hanches, kosala ete ezala mpasi mpo na mobɛli kotombola maboko na ye likolo ya mapeka na ye mpo na kolata to kotelema uta na kiti ya nse kozanga kosalela maboko na ye.
  • GCA systémique "Silent" occulte (Fièvre d'origine inconnu): Pene na 10 à 15 pour cent ya ba malades ya GCA bazalaka na zéro mpasi ya mutu to tendresse ya loposo ya mutu mpe ba présenter na esika na yango na ba semaines ya fièvres ya mokolo na mokolo oyo ekoki kolimbolama te, ba suki ya butu oyo ezo drencher, anorexie profonde, 5 à 10 kilos (11 à 22 livres) ya kokita ya kilo na nko te, mpe anémie normocytique, to ba présenter na aveugle "occult" ya mbalakaka sans douleur na liso moko accompagné kaka na ESR mpe CRP ya lola.

5. Mikakatano ya misisa minene mpe ya misisa ya bɔɔngɔ (AVC mpe Dissection Aortique) .

  • AVC ischémique vertébrobasilaire na carotid (3% à 7%): GCA alingaka ba segments extradural ya ba artéries vertébrales esika ba percer dura (po lamine élastique ezalaka dense kuna). Tala tango nionso bilembo ya AVC ya circulation postérieure (vertigo, ataxie, dysarthrie, dysphagie, hémianopie homonyme).
  • Sténose subclavienne/axillaire ya ba vaisseaux minene mpe aneurisme aortique thoracique: Na 25 à 50 pour cent ya ba malades ya GCA, imagerie PET-CT to CTA elakisaka inflammation ya aorta thoracique, ba artéries subclaviennes, mpe ba artéries axillaire, kosala claudication ya loboko tango ekomi likolo ya mutu, tansion asymétrique entre gauche mpe maboko ya mobali (> 10 à 15 mmHg différence), kobungisa pulse radiale, to retardé aneurisme aortique thoracique mpe dissection.

Bokeseni ya AION ya Artéritique (GCA) na AION oyo ya Artéritique te (NAION) .

Lokola Neuropathie optique ischémique antérieure non artéritique (NAION) ezali mpenza mingi koleka Artérite Cellule géante na mikóló ya mbula ya katikati mpe ya mikóló (eutaka na hypothénormose ya butu mpe bozindi ya microvasculaire na disque optique ya moke na esika ya vasculite), minganga ya miso mpe minganga ya urgence basalelaka tableau oyo ya bokokanisi ya mbuma mingi mpo na kokesenisa bango mibale mbala moko:

Ezaleli ya Clinique & Diagnostic AION ya misisa ya nzoto (A-AION mpo na Artérite Cellule Générale) . AION oyo ezali na Artérite te (NAION Infarctus microvasculaire) .
Bokabolami ya mibu Bobele mbula \(\ge 50\text{ mbula}\) (moyenne ya mbula 73 kino 76 ans; rare entre 50 na 60). Mingimingi mbula 50 kino 70 ans (moyenne ya mbula 60 ans; ekoki kosalema na 40s).
Ba Conditions Systèmes Associées Polymyalgie rheumatique (PMR), mpasi ya motó, mpasi ya loposo ya motó, claudication ya mbanga, fièvre, kokita ya kilo. Hypertension, diabète mellitus, hyperlipidémie, apnée obstructive ya pongi, ba meds ya tansion ya butu, to ba inhibiteurs ya PDE-5 (sildenafil/tadalafil).
Bokasi ya kobungisa komona Catastrophique: Koleka 60% à 70% présent na acuité visuelle mabe koleka 20/200 tii na Counting Fingers to No Light Perception (NLP). Moderate à Grave: Koleka 50% ba garder acuité visuelle malamu koleka 20/200 (mbala mingi 20/40 à 20/100) na défaut ya champ altitude inférieur.
Bilembo ya likebisi liboso ya kokufa miso Ezali liboso na amaurosis fugax (25%) to diplopie transitoire (15%) na mikolo liboso. Pene na ata mokolo moko te liboso ya amaurose fugax to diplopie; mobeli typiquement a découvrir perte ya vision sans pasi tango alamuki na tongo.
Emonani ya disque optique (Liso oyo ezo affecter) Édème ya disque optique palide"blanc-craie" (ezali pâle makasi mpe evimbaki kobanda mokolo ya 1), mbala mingi na ba taches ya coton-laine to occlusion cilioretinale concurrente. Édème sectoriel ou diffuse ya disque optique hypérémique (rose/rouge) na ba hémorragie ya mike mike oyo ezali na forme ya flamme.
Emonani ya Disque Optique (Liso ya Moninga ya Santé) Ratio physiologique normal ya kopo na disque (\(0.3\text{ na }0.5\)). "Disc-at-Risk": Disque optique ya moke, oyo etondi na bato na kopo ya physiologique ya moke to oyo ezali te (\(\le 0.1\)) esika ba fibres nerveuses ekangami makasi.
Ba marqueurs ya laboratoire inflammatoire (ESR & CRP) Emati makasi na > 90% kino 95%: ESR mbala mingi 50 kino 120+ mm/hr mpe CRP emati makasi, bakisa thrombocytose réactive (plaquettes > 400.000/\(\mu\)L). ESR normal, CRP normal, mpe nombre normal ya ba plaquettes pona âge.
Risk na Liso ya Mibale (Moninga) LIKAMA YA MAKASI: 30% kino 50% babungisaka miso na liso ya mibale na kati ya mikolo 1 kino 7 soki ba stéroïdes ya dose makasi ebandi mbala moko te. Risque aigu ya moke: Pene na 15% ya risque na mibu 5 (na mikolo te); ba corticostéroïdes epekisaka na ndenge ya kotyela motema te baninga ya miso NAION.
Angiografie na fluorescéine Remplissage choroïdale retardé makasi to absent pembeni ya ischémie ya disque optique. Remplissage choroïdale normal (kaka microcirculation ya disque optique nde ebebaka).

Workup ya diagnostic mpe protocole ya traitement ya Département d’urgence

+-------------------------------------------------------------------------+
|     EMERGENCY DEPARTMENT & RHEUMATOLOGY/OPHTHALMOLOGY GCA PROTOCOL      |
+-------------------------------------------------------------------------+
|  STEP 1: IMMEDIATE BLOOD DRAW (DO NOT WAIT FOR RESULTS TO TREAT)        |
|  * Erythrocyte Sedimentation Rate (ESR / Westergren method)             |
|    - Age-adjusted upper limit: Men = Age / 2; Women = (Age + 10) / 2    |
|    - In active GCA, ESR is typically > 50 mm/hr (often 70 to 120 mm/hr) |
|  * C-Reactive Protein (CRP - more sensitive than ESR; doesn't vary      |
|    with anemia/red cell shape; combined ESR + CRP sensitivity is 97%)   |
|  * Complete Blood Count (CBC): Check for normocytic anemia &            |
|    REACTIVE THROMBOCYTOSIS (Platelets > 400,000/mcL is highly specific) |
+-------------------------------------------------------------------------+
|  STEP 2: IMMEDIATE EMPIRIC CORTICOSTEROID THERAPY (MINUTE 0)            |
|  * IF VISUAL LOSS, AMAUROSIS FUGAX, DIPLOPIA, OR JAW CLAUDICATION:      |
|    ---> Give IV Methylprednisolone 500 mg to 1,000 mg IV over 60 min    |
|         immediately in the ER, repeated daily for 3 days, then switch   |
|         to Oral Prednisone 1 mg/kg/day (60 to 80 mg/day).               |
|  * IF HEADACHE / PMR ONLY (NO VISUAL OR ISCHEMIC CRANIAL SYMPTOMS):     |
|    ---> Give Oral Prednisone 40 to 60 mg (1 mg/kg) PO immediately.      |
+-------------------------------------------------------------------------+
|  STEP 3: CONFIRMATORY IMAGING & TEMPORAL ARTERY BIOPSY (DAYS 1 TO 14)   |
|  * Color Doppler Ultrasound of Temporal & Axillary Arteries:            |
|    - Looks for the dark non-compressible edematous wall "HALO SIGN"     |
|  * Temporal Artery Biopsy (Gold Standard Histopathology):               |
|    - Harvest >= 1.5 to 2.0 cm specimen (because GCA has "SKIP LESIONS"  |
|      where normal artery segments alternate with inflamed segments)     |
|    - Biopsy stays positive for 2+ weeks AFTER starting steroids         |
|  * Steroid-Sparing Biologic: Add Tocilizumab (IL-6 receptor antagonist, |
|    162 mg SC weekly, FDA/EMA approved per GiACTA trial) to prevent      |
|    relapse while tapering prednisone + PPI + Calcium/Vitamin D/Bisphos. |
+-------------------------------------------------------------------------+

Mpo na nini bolai ya biopsie ya artérie temporelle (\(\ge 1.5\text{ kino }2.0\text{ cm}\)) ezali na ntina: Phénomène ya "Skip Lesion".

Artérite ya baselile minene epelisaka bolai mobimba ya misisa ya motema ndenge moko te; na esika na yango, e attaquer na ba segments patchy, discontinues oyo babengaka "skip lesions," esika 5 millimètres étirement ya artérie inflamée makasi na ba cellules géantes multinucléées mpe lamina élastique fragmentée efandi kaka pembeni ya 5 millimètres étirement ya mur ya artérie entièrement normal. - Yango wana, ntango monganga ya lipaso ya misisa, monganga ya miso, to monganga ya matoi asali biopsie superficielle ya artérie temporelle (procédure ya miniti 30 oyo ezali na likama te na anesthésie locale pembeni ya ligne ya suki na ngambo oyo ezali na bilembo), malako esɛngaka kobuka eteni ya misisa ya misisa ata 1,5 kino 2,0 centimètres ya bolai (nsima ya fixation formaline rétrécissement) mpe kotala ba sections transversales sérieuses ebele mpo ba lésions ya saut ezala kozanga te. - Ata soki biopsie ya artérie temporelle ezongi négatif (oyo esalemaka na 10 à 15 pour cent ya ba cas ya solo ya GCA mpo na ba lésions ya skip to implication purement extracrânienne/postérieure ciliaire), biopsie négative ezo superposer jamais présentation clinique classique na ESR/CRP eleki mpe perte visuelle; minganga bazali kokoba kozwa lisalisi ya corticostéroïde mpe tocilizumab mobimba.

Echographie vasculaire ya mbangu ("Halo Sign") mpe IRM ya résolution ya likolo

Ba centres mingi ya sika ya rhumatologie mpe ya urgence esalaka sikoyo echographie duplex couleur rapide ya ba artéries temporelles superficielles (ezala ba branches frontales mpe pariétales) mpe ba artéries axillaires na kati ya 24 à 48 heures ya présentation: - Édème granulomatose actif ya mur artériel ebimisaka bague ya molili (hypoéchoïque), circonférentiel caractéristique autour ya lumen ya flux ya makila Doppler ya couleur oyo babengaka "Signe Halo," oyo elongi te ko plat tango sonde ya ultrasons e presser na se na loposo (positive « Signe de compression"). - Na EULAR mpe ACR guidelines, signe ya halo ya ultrasons temporal/axillaire ya malamu na mobeli oyo azali na probabilité clinique ya likolo endimisi diagnostic ya GCA na spécificité ya likolo, nzokande IRM ya mur ya misisa ya motó ya 3-Tesla ya résolution ya likolo to FDG-PET/CT ya nzoto mobimba ekoki ko détecter aortite ya misisa minene mpe artritis subclavian.

Script ya communication exacte pona triage ya lopitalo

Ntango okokóma na departema ya lisalisi ya nokinoki ya lopitalo elongo na mokóló moko oyo azali mokóló oyo azali na bilembo ya komona to bilembo ya kala ya motó ya artérite ya baselile minene, tángá script oyo mbala moko epai ya infirmier ya triage mpe monganga ya nokinoki:

"Nazali awa na mobeli moko ya mbula [Age] oyo akolisaki na mbalakaka [kobungisa komona / kokita ya mokuse ya komona / komona mbala mibale] na liso [ya gauche/droite] lelo, liboso na [motu mpasi ya sika ya tempelo / mpasi ya loposo ya motó ntango azali kosukola nsuki / mpasi ya kolɛmba ya mbanga nsima ya kotafuna bilei na boumeli ya miniti moko]. Tozali ko suspecter makasi Arteritis Cellules Giantes (Artéritis Temporal) na acute Risque ya neuropathie optique ischémique Lokola 30 à 50 pour cent ya ba malades oyo bazwaki traitement te bakendaka libela na libela na liso na bango ya mibale na mikolo, ba guidelines ya ACR na EULAR e recommandé mbala moko dose ya likolo ya méthylprednisolone intraveineuse (500 à 1.000 mg IV) kaka sikoyo na salle d'urgence AVANT ya kozela ba résultats ya makila ya ESR na CRP to biopsie temporelle ya artérie monganga ya lisalisi ya nokinoki atalelaka moto ya maladi mbala moko."

Liste ya vérification ya drapeau rouge mpo na ba malades oyo bazali na Polymyalgie Rheumatica (PMR) to ba stéroïdes coniques

Soki yo to moto moko ya libota oyo azali mokóló basilá kozwama na maladi ya Polymyalgie Rheumatica (PMR) to ozali sikawa kokitisa prednisone oyo ezwamaka na monɔkɔ mpo na Arteritis Cellules géantes, kangá liste oyo ya kosala makambo na frigo na yo mpe zonga mbala moko na salle d’urgence soki eloko moko esalemi:

  • Kokita nyonso ya mokuse, bozindo, mpela, to rido likolo ya liso moko (ata soki esili kopɛtola nsima ya miniti 2).
  • Episode nionso ya double vision (komona bilili mibale pembeni to likolo ya moko na mosusu), ata soki ya mwa ntango moke.
  • Kolɛmba ya sika, mpasi, to kolɛmba na misisa ya mbanga to na monoko ntango ozali kotafuna bilei.
  • Kozonga ya mpasi ya motó to mpasi ya tempelo ntango ozali kosimba loposo ya motó to kolata ba lunettes.
  • Kotika to koleka ata moke te ba dose ya prednisone to tocilizumab oyo okomeli na mbalakaka: Kotika ba stéroïdes na mbalakaka na tango ya GCA oyo ezali kosala ekoki kobimisa ezala mbala moko kozonga ya aveugle mpe crise surrénale aiguë oyo ezali na likama ya bomoi (tala ya biso guide d'urgence ya crise surrénale).

Zwá cours moko

Kozwa cours ya secours ya liboso ya maboko oyo endimami na Croix-Rouge, Ambulance ya St John, to mopesi ya formation ya urgence ya mboka ebongisaka yo mpo na koyeba ba urgences ya mbalakaka ya neurologique mpe vasculaire, kotala ba drapeaux rouges visuels mpe ya AVC, mpe kotombola malamu mpo na soins noki na lopitalo.

Mituna oyo batunaka mingi

Mpo na nini minganga bapesaka ba dose minene ya stéroïdes intraveineuses mpo na Artérite Cellule géante liboso ya kosala biopsie ya artérie temporelle?

Na maladi ya Artérite Cellule géante, soki makila oyo ekɔtaka na misisa ya miso ekangami na liso ya liboso, komonana na liso yango ebungaka pene na libela, mpe soki basalisi yango nokinoki na stéroïdes, bato 30 kino 50 likoló na monkama bakobungisa komona na liso na bango ya malamu oyo etikali na boumeli ya ngonga 24 tii mikolo 7. Kobanda dose makasi ya méthylprednisolone na misisa ekangaka mbala moko inflammation vasculaire mpo na kobikisa liso ya mibale, mpe ba pathologistes bakoki kaka koyeba polele inflammation ya mur artériel na biopsie ya artérie temporelle oyo esalemi kino poso 2 sima ya kobanda ba stéroïdes.

Ndenge nini nakoki koyeba bokeseni kati na claudication ya mbanga ya Artérite Cellule géante mpe mpasi ya matonga ya mbanga ya TMJ ordinaire to mpasi ya mino?

Trouble ya articulation temporomandibulaire (TMJ) mpe ba infections ya mino epesaka mpasi makasi mbala moko na ngonga oyo ozali koswa makasi, kofungola monoko na yo monene, to kobɛta mino moko boye, mbala mingi elongo na litonga ya mbanga oyo ezali kofina. Claudication ya mbanga oyo euti na Arteritis Cellules géantes ezali problème ya alimentation ya makila ya artérielle kaka lokola angina ya motema: mwa koswa na yo ya liboso ya bilei eyokaka malamu mpenza, kasi nsima ya miniti 1 to 2 ya kokoba kotafuna (mingimingi misuni to mampa ya croustillant), misisa ya mbanga na yo ekómaka mpasi makasi, ya kolɛmba mpe kolɛmba oyo etindaka yo otika kotafuna, mpe mpasi yango esilaka nsima ya miniti 1 to 2 ya kopema.

Moto oyo azali na mbula koleka 50 te akoki kozwa Arterite Cellulaire Générale (Artérite Temporal)?

Artérite cellulaire géante esalemaka pene na mikóló ya mbula 50 mpe koleka (na mwayene ya mbula 72 tii 76) mpo réaction auto-immune ebandisami na mbongwana ya structure oyo etali mbula na ba couches élastiques ya ba artéries moyennes mpe ya minene. Ntango elenge mokóló oyo azali na nse ya mbula 40 azwi inflammation granulomatose ya misisa minene ya aorta mpe ya bitape ya carotide to ya subclavien, ezali maladi oyo ezali na boyokani na yango kasi ekeseni oyo babengaka Takayasu arteritis ("maladi sans pulsation").

Biopsie ya artérie temporelle négative elingi koloba nini soki monganga na ngai azali kaka kokanisa ete nazali na Artérite ya Cellule géante?

Lokola Artérite ya Cellule géante e attaquer ba artéries na ba "skip lesions" ya ba patchies (kotikaka ba étirement normales ya artérie entre ba étirement inflamés) to ekoki ko inflamar na ndenge ya kopona ba artéries ciliaires ya sima sima ya liso tout en épargner etape ya elongi, 10 à 15 pour cent ya ba malades oyo bazali vraiment na Artérite Cellule géante bakozala na échantillon ya biopsie normale. Yango wana, soki bilembo na yo, mbula na yo, mpe ba tests ya makila ya ESR/CRP oyo emati to "elembo ya halo" ya ultrasons elakisaka makasi Artérite Cellule géante, minganga bakokoba traitement ya stéroïdes mobimba mpo na kobatela bomoni na yo atako biopsie négative.

Ba sources e vérifié

  • Collège américain ya rhumatologie (ACR) mpe Fondation ya vasculite (Etats-Unis): Fundation ya Collège américain ya Rheumatologie/Vasculite Guideline ya 2021 mpo na gestion ya artérite cellulaire géante mpe artérite takayasu (Maz et al., Arthrite & Rheumatologie, epesi toli ya mbala moko ba glucocorticoïdes ya pulsation intraveineuse na dose makasi [méthylpredsolonine 500 kino 1.000 mg/jour] mpo na GCA na perte aiguë ya miso to fugax ya amaurose liboso ya biopsie, bakisa mpe kobakisa tocilizumab).
  • Alliance européenne des associations pour l’héumatologie (EULAR): Makanisi ya EULAR mpo na gestion ya vasculite ya misisa ya minene mpe Makanisi ya EULAR mpo na kosalela imagerie na vasculite ya misisa ya minene na pratique clinique (echographie temporal/axillaire rapide "signe halo," initiation immédiate ya stéroïdes, mpe \(\ge 1.5\text{ cm}\) bolai ya biopsie sima ya fixation).
  • Société britannique ya rhumatologie (BSR) mpe Royal College of Ophthalmologists (Royaume-Uni): Likambo ya Société britannique mpo na rhumatologie mpo na diagnostic mpe traitement ya artérite cellulaire géante (Mackie et al., banzela ya méthylprednisolone intraveineuse ya urgence mpe différenciation ya A-AION vs. NAION).
  • New England Journal of Medicine (GiACTA Trial): Bomeki ya Tocilizumab na Artérite ya ba cellules géantes (Stone et al., oyo ezali kolakisa rémission soutenue sans glucocorticoïdes na blocage ya récepteur IL-6 sous-cutané poso na poso).

Ba guides oyo etali yango