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Arteritis by'agateganyo (Arteritis Giant Cell), Kubabara Urwasaya Iyo Uhekenya, no Gutakaza Icyerekezo gitunguranye

Imibare yihutirwa itandukanye nigihugu. Uru rupapuro rukoresha 911, umubare kuri Amerika na Kanada. Ibindi bihugu byinshi bikoresha 112, 999, cyangwa undi mubare. Shakisha umubare wigihugu cyawe.

Kubabara umutwe mushya kurusengero rumwe cyangwa byombi, ubwuzu bwumutwe mugihe wogeje umusatsi wawe, cyangwa ububabare bwurwasaya mugihe uhekenya ibiryo kumuntu mukuru ufite imyaka 50 cyangwa irenga ni ibintu byihutirwa byubuvuzi bizwi nka ** Temporal Arteritis ** cyangwa ** Giant Cell Arteritis (GCA) . Iyo iyi autoimmune nini-nini ya vasculitis yaka kandi igafunga amashami yimitsi ya ophthalmic na short posterior ciliary arteri igaburira imitsi ya optique, itera ubuhumyi butunguranye, butababara, budasubirwaho mumaso imwe ( Arteritic Anterior Ischemic Optic Neuropathie [A-AION] **). Ndetse byihutirwa cyane, ijisho rya mbere rimaze gutakaza icyerekezo, ** 30 kugeza 50% byabarwayi batavuwe bahuma buhumyi mumaso yabo ya kabiri (asigaye) mumasaha 24 kugeza kumunsi 7 **. Niba wowe cyangwa umuntu ufite imyaka 50 cyangwa irenga ufite iterambere ryumutwe mushya wurusengero, kurwara urwasaya mugihe uhekenya, iyerekwa kabiri, kutabona neza, cyangwa kubura icyerekezo gitunguranye, hamagara numero yawe yihutirwa ako kanya ukoresheje nimero yihutirwa. Ongera usuzume imitsi ijyanye na neuro-ophthalmic byihutirwa muri ocular stroke hamwe na retinal retinal arteri, gutakaza icyerekezo gitunguranye, gutandukana kwa retina, no gufunga inguni glaucoma, na kumenyekanisha inkorora no gutabara byihutirwa.

Kora ubu

  1. ** Hamagara nimero yawe yihutirwa cyangwa ujye mubitaro byihutirwa byihutirwa ** (911 muri Reta zunzubumwe zamerika na Kanada, 112 hirya no hino mubumwe bw’ibihugu by’Uburayi, 999 mu Bwongereza) niba umuntu mukuru ufite imyaka 50 cyangwa irenga akuze ** Guhindura amashusho ** Ntuzigere utegereza umuganga w'amaso, umuganga w'amenyo, cyangwa gahunda y'ibanze y'icyumweru gitaha.
  2. ** Gerageza buri jisho ukwacyo ubungubu utwikiriye ijisho rimwe icyarimwe ukoresheje ikiganza cyawe. ** Abarwayi akenshi ntibamenya ko ijisho rimwe ryatakaje kimwe cya kabiri cyangwa amaso yacyo yose kugeza bapfutse ijisho ryiza. Andika isaha niminota nyayo iyerekanwa igaragara, igicucu, cyangwa iyerekwa rya kabiri ryatangiye.
  3. ** Erekana ako kanya kuri "Ibendera ritanu rya Cranial na Sisitemu Ibendera ritukura rya selile Arteritis": **
  4. ** Urwasaya cyangwa ururimi rwamagambo: ** Urwasaya, umusaya, cyangwa ururimi bitangira kubabara, kubabara, cyangwa kumva unaniwe nyuma yo guhekenya ibiryo byumye (nk'inyama cyangwa umutsima) amasegonda 60 kugeza kuri 90, bikaguhatira guhagarara ukaruhuka?
  5. ** Ubwuzu bwo mu mutwe: ** Kwoza umusatsi wawe, kwambara amadarubindi cyangwa ingofero, cyangwa gushyira umutwe wawe ku musego wumva ubabaye cyangwa wakomeretse hejuru y'urusengero rwawe cyangwa ikamba?
  6. ** Imitsi idasanzwe yigihe gito: ** Ese imiyoboro iri imbere yugutwi kwawe no kuruhande rwurusengero rwawe irasa nkigaragara cyangwa yuzuye, wumva ufite ubwuzu cyangwa umugozi umeze nkugukoraho neza, cyangwa kubura impyiko zisanzwe?
  7. ** Ibimenyetso byigihe gito byo kuburira: ** Wigeze ugira igice kigufi cyiminota 2 kugeza kuri 15 yimyenda yumukara cyangwa umwijima mumaso imwe (** amaurose fugax **) cyangwa igice kigufi cya ** icyerekezo cya kabiri (diplomopiya) ** muminsi yashize?
  8. ** Polymyalgia Rheumatica (PMR) no gutwika sisitemu: ** Wigeze ugira ubukana bwa mugitondo ukababara ku bitugu byombi, ijosi, no mu kibuno bimara iminota irenga 45, umuriro udasobanutse wo hasi, ibyuya nijoro, cyangwa gutakaza ibiro utabigambiriye?
  9. ** Bwira umuforomo n’umuganga w’icyumba cyihutirwa ukimara kuhagera: "Turakeka ko Arteritis ya Giant Cell Arteritis (Temporal Arteritis) kandi dukeneye isuzumabumenyi ryihuse kuri static-dose corticosteroide kugirango irinde iyerekwa."
  10. ** Shimangira ko kuvura corticosteroide bidatinda mugihe utegereje gahunda ya biopsy yigihe gito cyangwa gahunda ya ultrasound. biopsy ikomeza kwisuzumisha muminsi 10 kugeza 14 nyuma ya steroid itangiye, mugihe gutegereza amasaha 24 kuri biopsy idafite steroyide bishobora guhuma amaso asigaye ubuziraherezo.
  11. ** Ntugafate ibinini byongera umuvuduko wamaraso cyangwa vasodilatrice mbere yuko ibitaro bigera.

Danger

Iyo Arteritis nini ya selile imaze gutera amaso mu jisho rya mbere, iryo jisho rya mbere ntirishobora gukira neza ndetse no kuvurwa. Inshingano zose zihutirwa muri Temporal Arteritis hamwe nibimenyetso bigaragara ni ** gukiza ijisho mugenzi wawe (wa kabiri) guhuma amaso mumasaha 24 kugeza 168 ari imbere **. Isaha yose irengana idafite methylprednisolone yuzuye cyane (miligarama 500 kugeza 1.000 miligarama IV kumunsi) isiga imitsi ya optique ya kabiri ihura nindwara zitunguranye, zidasubirwaho.

Igiti cyemezo: Tage na steroid byihutirwa mukekwaho kuba arterite nini ya selile

flowchart TD
    Start["Imyaka Yabakuze> = Imyaka 50 Numutwe Mushya, Ububabare bwumutwe, Ububabare bwo mu rwasaya, PMR, cyangwa Guhinduka Kuboneka"] --> VisionCheck{"Hoba hari ibimenyetso biboneka bihari? ."}

    VisionCheck -->|"YEGO: Gutakaza Amashusho, Dimming Yigihe gito, cyangwa Diplopiya Yubu"| VisualEmergency["OPHTHALMIC VASCULITIS YIHUTIRWA (KIZA IJISHO RYA KABIRI): 1. Hamagara 911/112/999 -> Ishami ryihutirwa ryibitaro NONAHA. 2. Administer Stat High-Dose IV Methylprednisolone (500 kugeza 1.000 mg IV / umunsi x iminsi 3) VUBA mbere ya biopsy. 3. Shushanya Stat ESR, CRP, & CBC (Platelets) kumwanya wa IV."]

    VisionCheck -->|"NTA bimenyetso bigaragara"| JawIschemiaCheck{"Ese Jaw Claudication, Indimi, cyangwa Limb / Cranial Ischemia Irahari?"}

    JawIschemiaCheck -->|"YEGO: Ububabare bw'urwasaya / Ururimi Iyo uhekenya (Ibyago byinshi byo guhuma amaso)"| HighRiskCranial["HANI-RISK CRANIAL GCA: 1. Isuzuma ryishami ryihutirwa UYU MUNSI. 2. Tangira Umuvuduko mwinshi wo mu kanwa Prednisone (60 mg / kumunsi, cyangwa 1 mg / kg / kumunsi) cyangwa IV Methylprednisolone ako kanya. 3. Byihuta Byihuta-Gukurikirana by'agateganyo Arteri Ultrasound + Biopsy muminsi 7."]

    JawIschemiaCheck -->|"OYA Urwasaya"| ClassicCranialCheck{"Urusengero rushya rwaho / ububabare bwumutwe, ubwuzu bwumutwe, umubyimba wijimye / udafite umuvuduko wigihe gito, cyangwa PMR igitugu / gukomera ikibuno + cyazamuye ESR / CRP?"}

    ClassicCranialCheck -->|"YEGO: Igikorwa gikomeye kitagoranye Cranial cyangwa Kinini-Vessel GCA"| StandardGCA["GUKORA CRANIAL GCA NTA ISHEMIYA: 1. Tangira Dose Yinshi Yumunwa Prednisone (40 kugeza 60 mg / kumunsi) VUBA uyumunsi (ntuzategereze biopsy). 2. Gahunda Yigihe gito & Axillary Artery Ultrasound ("Halo Sign") na / cyangwa Arteri yigihe gito Biopsy muminsi 7 kugeza 14. 3. Reba Tocilizumab steroid-sparing therapy."]

    VisualEmergency --> BiopsyFollow["Kwemeza Gusuzuma Nyuma ya Steroide Yatangiye: Kora by'agateganyo / Axillary Ibara Doppler Ultrasound + Biopsy yigihe gito (byibura cm 1,5 kugeza kuri 2.0 z'uburebure nyuma yo gukosorwa) muminsi 10 kugeza 14."]

Pathophysiology: Ukuntu autoimmune granulomatous vasculitis ihuma amaso

Impamvu ingirabuzimafatizo ya Arteritis yibasira imiyoboro mito nini nini ya elastique nyuma yimyaka 50

** Indwara nini ya Arteritis (GCA) **, amateka yiswe ** Arteritis Temporal Arteritis ** cyangwa ** Indwara ya Horton **, ni vasculite yibanze ya sisitemu ikunze kugaragara mubantu bakuze mubihugu byiburengerazuba, ibaho ** hafi yabantu bafite imyaka 50 nayirenga ** (bafite ikibazo cyo hejuru cyane hagati yimyaka 70 na 80, kandi ikagira ingaruka kubagore inshuro 2 kugeza kuri 3 kurenza abagabo).

Bitandukanye na vasculitis ntoya, igitero cyubudahangarwa muri GCA cyibasiye ** imiyoboro mito nini nini nini ifite lamina y'imbere ya elastique imbere, cyane cyane amashami ya ** adasanzwe yinyuma ya arteri yo hanze ninyuma **, imitsi ya vertebral **, ** subclavian na axillary ** **, na ** thoracic aorta **: 1. ** Gukora Akagari ka Dendritic muri Adventitia: ** Impinduka zijyanye n'imyaka murukuta rwa arterial hamwe nibidukikije bitamenyekana bikora ingirabuzimafatizo ya dendritic selile iba mumbere yinyuma (adventitia) yimitsi ikoresheje reseptor zisa na Toll. 2. ** Kwinjiza CD4 + T-Akagari (Inzira ya Th1 na Th17): ** Ingirabuzimafatizo zikora zifata CD4 + umufasha T-lymphocytes binyuze muri * vasa vasorum * (imiyoboro mito yintungamubiri igaburira urukuta rw'imitsi). Izi T-selile zitandukanye mumaboko abiri atandukanye yo gutwika: - ** Ukuboko kwa Th17 / Interleukin-6 (IL-6): ** Itera umwijima sisitemu yumwijima wa poroteyine ikaze (C-reaction protein, fibrinogen, serum amyloide A), umuriro mwinshi, ibyuya nijoro, ibara ryinshi rya platine, na Polymyalgia Rheumatica; uku kuboko kwumva neza corticosteroide na ** IL-6 yakira reseptor (tocilizumab) . - ** Th1 / Interferon-gamma (IFN - $ gamma $) Ukuboko: ** Itwara macrophage fusion muri ** selile nini nini nini ** yonona lamina y'imbere kandi ikarekura ibintu bikura bikomoka kuri platine (PDGF) hamwe niterambere ryimitsi (VEGF). 3. ** Hyperplasia yibanze cyane (Luminal Occlusion): ** Mu gusubiza PDGF na VEGF, myofibroblast yimuka imbere kandi ikwirakwira vuba imbere mumbere (intima) yimitsi. Urukuta rwa arterial rwabyimbye imbere kugeza umuyoboro wamaraso rwagati ugabanijwe kugeza kuri pinhole cyangwa ucomekwa rwose na trombus ( luminal occlusion **), inzara ibice byo munsi ya ogisijeni.

Ni izihe miyoboro yihariye ya ocular igaragara muri GCA?

Iyo GCA yibasiye imiyoboro y'amaraso ya orbit, irerekana uburyo butangaje bwa anatomique kuri ** Short Posterior Ciliary Artery (SPCAs) **, ikurikirwa na ** Central Retinal Artery (CRA) ** na ** Ophthalmic Artery **: - ** 80 kugeza 85 ku ijana byo gutakaza intumbero ya GCA: Arteritike Yimbere Ischemic Optic Neuropathie (A-AION). Iyo GCA ikubiyemo imiyoboro migufi ya ciliary arteri, umutwe wa optic nervice uhura nindwara nini, yuzuye. Mu kizamini cya fundoscopique, disiki ya optique isa ** yabyimbye na "chalky yera" (pallid disc edema) ** aho kuba ibara ryijimye gusa, kuko amaraso ya zeru aguma muburiri bwa capillary. - ** 10 kugeza kuri 15 ku ijana byo gutakaza icyerekezo cya GCA: Arteritike yo hagati ya Arteritike yo hagati (A-CRAO). - ** 5 ku ijana byo gutakaza intumbero ya GCA: Arteritic Posterior Ischemic Optic Neuropathie (A-PION) cyangwa Cilioretinal Artery Occlusion. kugeza ibyumweru 6 nyuma.

Kuberiki iyerekwa ryigihe gito (diplomopiya) na amaurose fugax niburira amasaha 24 yo kubara

Abagera kuri ** 30 kugeza 40 ku ijana by'abarwayi bahumuka burundu bava mu ngirabuzimafatizo ya Arteritis bahuye n'ibimenyetso byo kuburira iminsi 1 kugeza 7 MBERE yuko umwijima wabo uhoraho ** wabuze cyangwa wirukanwa: 1. ** Gutakaza Monocular Vision Yatakaye (Amaurose Fugax muri GCA): ** Bitandukanye na emburike ya amurose fugax ivuye ku cyapa cya karotide (aho kristu ya cholesterol icomeka icyombo mu minota 5), ibara ryigihe gito cyangwa imvi zijimye muri GCA bibaho kubera ko umuvuduko wamaraso unyuze mugice gito cya arterière arteri. Gusa uhagaze ku ntebe, wunamye, cyangwa uhura n'umuvuduko ukabije w'amaraso wijoro ugabanuka ugabanya imitsi ya optique munsi yumuryango muminota 5 kugeza kuri 20. 2. ** Diplopiya yinzibacyuho cyangwa idahwema (Double Vision, iboneka muri 10 kugeza kuri 15 ku ijana bya GCA): ** Imitsi ya ** idasanzwe idasanzwe ** yimura ijisho ryijisho (hamwe n imitsi ya cranial III, IV, na VI ibayobora) igaburirwa namashami yimitsi yimitsi y'amaso. Iyo GCA igabanije imitsi yimitsi, imitsi imwe cyangwa myinshi yijisho iba ischemic, bigatera gutambuka gutunguranye cyangwa guhagaritse ** icyerekezo cya kabiri ** gikunze guhindagurika kumunsi. ** Mu murwayi uwo ari we wese ufite imyaka 50 cyangwa irenga, iyerekwa rishya ni Arteritis nini ya selile kugeza igihe bigaragaye ukundi ** kandi bikunze kubanziriza ubuhumyi bwuzuye bwamaso amasaha 24 kugeza 72.

Kumenya amavuriro yihishe ya Arteritis y'agateganyo

Kubera ko Arteritis nini ya selile ikongeza amashami menshi ya karotide yo hanze, karotide y'imbere, vertebral, na aortic arche, abarwayi bahora berekana mbere kubaganga b'amenyo **, amavuriro yizuru-izuru-umuhogo, amavuriro, cyangwa abaganga rusange ** aho kuba abaganga b'amaso. Kumenya ibi bigana byigana bikiza kureba nubuzima:

1. Kwinyoza amenyo & TMJ Mimic: Urwasaya rwo mu rwasaya, Kuvuga ururimi, no kubabara amenyo.

** Urwasaya claudication ** ruri hafi ya ** 45 kugeza kuri 50% byabarwayi barwaye GCA ** kandi rufite igipimo cyiza cya ** cyiza cyane (umwihariko wo kwisuzumisha) ** mubimenyetso byose byamavuriro yo guhanura biopsy nziza yigihe gito: - ** Impamvu ya Anatomique: ** Kugabanuka k'umubyimba wa ** maxillary arteri **, ** arteri yo mumaso **, na ** arteriire yigihe gito ** (amashami yimitsi yo hanze ya karotide) itanga amaraso kumitsi ya ** masseter, temporalis, na pterygoid yo kwikinisha **. - ** Nigute Twatandukanya GCA Jaw Claudication nindwara ya TMJ cyangwa Ibibyimba by amenyo: **

Ikiranga ivuriro Ingirabuzimafatizo nini ya Arteritis (Urwasaya / Ururimi) Igihe gito (TMJ) Imikorere idahwitse / Arthritis Kuvura amenyo / Neuralgia Trigeminal
** Igihe Cyukuri Cyububabare Mugihe cyo guhekenya ** ** Ububabare bwa zeru kurumwa bwa mbere. ** Birababaza ako kanya ukinguye umunwa mugari cyangwa urumye ** ku ncuro ya mbere cyane y'ibiryo bikomeye; biherekejwe no gukanda byumvikana, pop, cyangwa gufunga kumatwi. ** Ibibyimba by'amenyo: ** Ububabare buhoraho bwiyongera ako kanya n'amazi ashyushye / akonje cyangwa gukubita iryinyo rimwe.
** Trigeminal neuralgia: ** Amasegonda 2-isegonda-inkuba yakubiswe no gukorakora byoroheje umusaya cyangwa koza amenyo.
** Ingaruka zo Guhagarika no Kuruhuka Urwasaya ** ** Kuruhuka byuzuye muminota 1 kugeza kuri 3 yo guhagarika guhekenya ** no kuruhuka umunwa. Kubabara ingingo zidakunze kumara amasaha menshi nyuma yo kurya cyangwa kunyoza amenyo nijoro. Ibibyimba by'amenyo bikomeza kumanywa nijoro; trigeminal neuralgia irasa muri paroxysms.
** Ururimi & Kumira Uruhare ** Irashobora gutera ** imvugo claudication ** (kubabara cyangwa gutwika umunaniro wururimi mugihe uvuga cyangwa urya), ** blanching / cyanose yururimi *, cyangwa ** ibisebe byirabura byirabura bikabije kuruhande rwururimi ** ( infarction y'ururimi * biva mu mitsi y'ururimi). Ntukigere utera ischemia y'ururimi, guhindagura ururimi, cyangwa necrosis yo mumutwe. Ntukigere utera ururimi cyangwa kuzamura ESR / CRP hamwe no gutakaza amaso.

2. Iburira rya Dermatologique: Ubwuzu bwo mu mutwe na Necrosis yo mu mutwe

  • ** Ubwitonzi bwo mu mutwe: ** Kuberako ** arterialial by'agateganyo, inyuma ya auricular, na octipital arteriire ** itanga ibice bya galeal na cutane yo mumutwe, ischemia yuruhu rwumutwe ituma kwiruka gusa ibimamara mumisatsi, gushira umutwe kumusego, cyangwa kwambara ibirahuri byo gusoma bikomeretsa bikabije.
  • ** Necrosis yo mu mutwe: ** Muri GCA itavuwe cyane, gufunga burundu imiyoboro y'agateganyo ndetse na arterière ituma ibibyimba by'uruhu ku nsengero cyangwa vertex y'umutwe bihinduka ibara ry'umuyugubwe, ibisebe, n'ibisebe bigahinduka eschar y'umukara (** scalp necrosis **), bikunze kwibeshya ku shitingi (herpes zoster). Kuberako necrosis yo mumutwe yerekana ischemia ikabije ya pan-cranial, abarwayi bafite necrosis yo mumutwe bafite ** barenga 60% byubuhumyi budasubirwaho **.

3. "Occult" Arteritis nini ya selile (20% yimanza zubuhumyi zifite umutwe wa Zeru)

Umutego ukomeye mubuvuzi bwihutirwa ni ugutekereza ko umurwayi udafite umutwe adashobora kugira Arterite yigihe gito. Ivuriro ryerekana ko ** hafi 20 ku ijana by’abarwayi barwaye biopsy yemejwe na Arteritis nini ya Arteritis bafite ubumuga bwo kutabona bafite "Occult GCA" **: bagaragaza kubura amaso atababara mu jisho rimwe na ** umutwe wa zeru, ubwonko bwumutwe wa zeru, hamwe na arteriire yigihe gito **, nkibimenyetso byoroshye bya sisitemu (umunaniro ukabije w'igitugu, umunaniro, ibiro bya CRP). Kubwibyo, ** buri murwayi wese ufite imyaka 50 cyangwa irenga yerekana hamwe na Anterior Ischemic Optic Neuropathie (AION) cyangwa Central Retinal Artery Occlusion (CRAO) agomba kuba afite statut ESR, CRP, hamwe na laboratoire yuzuye yamaraso (platelet) yashushanyije mubiro byihutirwa **.

4. Polymyalgia Rheumatica (PMR) Kwuzuzanya (50% by'abarwayi ba GCA)

** Polymyalgia Rheumatica (PMR) ** na Arteritis nini ya selile nini ihuza imiterere ikingira indwara imwe: - ** 40 kugeza 50 ku ijana by'abarwayi basuzumwe na Arterite nini ya selile ** bafite ibimenyetso bya PMR hamwe. - ** Abarwayi 15 kugeza kuri 20 ku ijana babanje gusuzumwa na PMR ** yitaruye (bafata prednisone nkeya, $ 10 inyandiko {kugeza} 15 inyandiko {mg / umunsi} \(, kubera kubabara, ibitugu bikaze, ijosi, n’umukandara wo mu kibuno mugitondo) nyuma baza kurwara Arteritis nini cyane. - ** Umuburo w'ingenzi ku barwayi ba PMR: ** Igipimo gito cya prednisone (\) 10 inyandiko {kugeza} 20 umwandiko {mg} $ buri munsi) bivura gukomera ku rutugu muri PMR ** ariko ntibikomeye bihagije kugira ngo birinde ubuhumyi indwara ya Arterite nini ya **. Umurwayi wese ufite PMR ufite uburibwe bushya bwumutwe, ubwonko bwumutwe, kubabara urwasaya mugihe cyo guhekenya, cyangwa guhumeka neza agomba guhita yitabaza ishami ryihutirwa kugirango yongere yivurwe cyane na GCA steroid ivura ($ 60 inyandiko {mg} $ umunwa cyangwa $ 1.000 inyandiko {mg} $ IV).

5. Kinini-Vessel GCA, Kwiba Subclavian, hamwe nubwonko bwubwonko

Muri 30 kugeza 80 ku ijana by'abarwayi ba GCA bashushanyije hamwe na PET-CT cyangwa CT angiography, granulomatous vasculitis irenze insengero zinjira muri ** aorta, subclavian, axillary, na arterière **: - ** Claudication yamaboko hamwe numuvuduko wamaraso utaringaniye: ** Kugabanuka kwimitsi ya subclavian na axillary itera umunaniro wamaboko iyo uteruye ibintu hejuru, amaboko akonje, impiswi ya radiyo idahari, hamwe numuvuduko ukabije wamaraso wa sisitemu ya $> 10 inyandiko {kugeza} 15 inyandiko {mmHg} $ hagati yamaboko yi bumoso n iburyo **. - ** Vertebrobasilar Brainstem na Cerebellar Stroke: ** Bitandukanye na aterosklerose (ifata imiyoboro y'amaraso), GCA yibasiye cyane imiyoboro y'amaraso ya ** idasanzwe yo mu ijosi mbere yuko izamuka mu ijosi mbere yo gutobora mater mater. Abagera kuri ** 4 kugeza kuri 7 ku ijana by'abarwayi ba GCA bafite ikibazo cyubwonko bukabije bwa ischemic , cyane cyane mubutaka bwa ** vertebrobasilar ** (bitera vertigo itunguranye, gutakaza uburimbane / ataxia, kuvuga nabi, dysphagia, cyangwa ubuhumyi bwimpande zombi). - ** Thoracic Aortic Aneurysm no Gutandukana: ** Abarwayi ba GCA bafite ibyago byikubye inshuro 17 17 bya thoracic aortic aneurysm na gutandukana aortic ugereranije nurungano ruhuye nimyaka, bisaba ibyingenzi hamwe nigihe cyo gufata amashusho ya aortic.

Kwipimisha amaraso byihutirwa no kwerekana amashusho mubitaro

1. Ibizamini bitatu byateganijwe byamaraso: ESR, CRP, na platine

Mbere cyangwa mugihe ushyira umurongo wimitsi ya methylprednisolone, abaganga byihutirwa bashushanya ibizamini bitatu byamaraso byihuse: 1. ** C-Intungamubiri za poroteyine (CRP): ** Ikimenyetso gikomeye cyane cyerekana icyiciro (cyazamuye $> 5 inyandiko {kugeza} 10 inyandiko {mg / L} $ muri ** 95 kugeza 98 ku ijana ** ya GCA itavuwe). Bitandukanye na ESR, CRP ntabwo igorekwa na anemia cyangwa imiterere ya selile itukura kandi irazamuka kandi igwa vuba hamwe nibikorwa byo gutwika. 2. ** Igipimo cya Erythrocyte Igipimo (Igipimo cya ESR / Westergren Igipimo): ** Kuranga hejuru cyane (akenshi ** $> 50 inyandiko {kugeza} 100+ inyandiko {mm / isaha} $ **) muri GCA. - * Nigute ushobora kubara imyaka isanzwe ihindurwa ntarengwa yo hejuru ya ESR: * Kubagabo, $ inyandiko {Max Normal ESR} = inyandiko {Imyaka} div 2 $. Kubagore, $ inyandiko {Max isanzwe ESR} = ( inyandiko {Imyaka} + 10) div 2 $. . 3. ** Kubara Amaraso Yuzuye (CBC) Urebye Byumwihariko kuri Platelets (Reaction Thrombocytose): ** Iyobowe nurwego rwo hejuru rwa Interleukin-6, ** reaction trombocytose (platelet ibara $> 400,000 / mu inyandiko {L} $) ** irahari hejuru ya 50% byimanza za GCA zikora kandi mubyukuri ni bihanura byindwara ya karande ya **.

Warning

** Ese ESR na CRP byombi birashobora kuba ibisanzwe muri biopsy yemejwe na Arteritis ya Giant Cell? ** Yego, hafi ya ** 2 kugeza kuri 4 ku ijana by'indwara ya GCA yanduye biopsy ** (cyane cyane ku barwayi basanzwe bafata steroyide nkeya kuri PMR cyangwa immunosuppressants), ESR na CRP byombi biri muri laboratoire isanzwe. Niba umurwayi ufite imyaka 50 cyangwa irenga afite claudication ya classique ya **, kubyimba pallid chalky optic kubyimba, cyangwa umugozi utanga isoko nkumuyoboro wigihe gito **, abaganga byihutirwa ** bagomba gutangira ako kanya corticosteroide ikabije ** nubwo ESR na CRP bagaruka mubisanzwe.

2. Ultrasound Yihuta-Yihuta ("Ikimenyetso cya Halo") na Biopsy Yigihe gito

Iyo corticosteroide ikabije itangiriye mu ishami ryihutirwa kugirango irinde umurwayi kureba, rubagimpande, indwara z’amaso, hamwe n’itsinda rishinzwe kubaga imitsi yemeza ko hasuzumwe hakoreshejwe uburyo bubiri bwuzuzanya:

  • ** Ibara rya Doppler Ultrasound ya Arteriire Yigihe gito na Axillary (Umurongo wa mbere mumabwiriza ya EULAR): **
  • Umuvuduko ukabije w'amaraso ultrasound probe isuzuma imiyoboro ibiri yigihe gito (imitsi isanzwe, imbere, n'amashami ya parietal) hamwe nimiyoboro yimitsi yombi.
  • ** "Ikimenyetso cya Halo": ** Indwara yumuriro imbere yurukuta rwa arterial igaragara nkimpeta yijimye, ** idacogora impeta ya hypoechoic ("halo") ikikije ibara Doppler maraso itemba lumen **.
  • ** Idirishya ryigihe cya Ultrasound: ** Bitandukanye nimpinduka za biopsy (zimara ibyumweru 2 bishize), Ikimenyetso cya ultrasound Halo gitangira kugabanuka byihuse iyo steroid nyinshi ya IV itangiye kandi ** irashobora kuzimira nyuma yiminsi 3 kugeza kuri 7 yo kuvura steroid **. Kubwibyo, ultrasound yihuta-yihuta igomba gukorwa mugihe cyamasaha 24 kugeza kuri 72 ** yo gutangira steroid.
  • ** Biopsy Yigihe gito (TAB, Gold Standard Histopathology): **
  • Yakozwe nkuburyo bwo kubaga bworoheje bwo kubaga munsi ya lidocaine anesthesia yaho, gusarura igice cyumuvuduko wigihe gito utagaragara kuruhande rwibimenyetso.
  • ** Impamvu Uburebure bwa Biopsy ($ ge 1.5 inyandiko {kugeza} 2.0 umwandiko {cm} $) Ibintu ("Skip Lesions"): ** Arteritis Giant Cell Arteritis yotsa imitsi mubice (** "skip lesions" **), bivuze ko milimetero 5 za arteriire zishobora kugaragara nkibisanzwe hafi ya milimetero 5 zumuriro ukabije. Kugira ngo wirinde ibinyoma-bibi bitandukanya icyuho gisukuye, abaganga basarura igice cya ** kitagizwe na arterial igice cya santimetero 2,2 kugeza kuri santimetero 2,5 (gipima byibuze santimetero 1.5 kugeza kuri 2.0 nyuma yo gukosorwa nyuma ya formine) ** hanyuma bagasuzuma ibice byinshi bikurikirana.
  • ** Idirishya ryigihe cya Biopsy: ** Granulomatous infiltrates, selile nini, hamwe no gucamo ibice imbere ya lamina yimbere bikomeza kugaragara neza munsi ya microscope byibura ** byibura iminsi 10 kugeza 14 (kandi akenshi bigera kumyumweru 4) nyuma yo gutangira corticosteroide **. ** Ntuzigere utinda gutangira steroid kugirango utegereze biopsy. **

Ibimenyetso bishingiye ku bitaro corticosteroid na protocole yo kuvura ibinyabuzima

Amabwiriza mpuzamahanga agezweho kuva muri ** American College of Rheumatology (ACR) **, ** Ihuriro ry’ibihugu by’i Burayi by’amashyirahamwe y’indwara ya rubagimpande (EULAR) **, na ** Umuryango w’Abongereza bashinzwe indwara ya rubagimpande (BSR) ** ugabanya ubuvuzi bwihutirwa ukurikije niba ischemia igaragara cyangwa ihari:

Kwerekana Ivuriro Byihutirwa Corticosteroid Regimen Guhuza & Steroid-Sparing Therapy
** GCA hamwe no Gutakaza Amashusho akomeye, Amaurose Fugax, cyangwa Icyerekezo cya kabiri (Diplopiya) ** ** Methylprednisolone yinjiza $ 500 inyandiko {kugeza} 1.000 inyandiko {mg} $ IV buri munsi muminsi 3 ikurikiranye ** mubitaro, hanyuma ihita ikurikirwa na ** Dose-Dose yo mu kanwa Prednisone $ 1 inyandiko {mg / kg / kumunsi} $ (mubisanzwe $ 60 inyandiko {kugeza} 80 inyandiko {mg} $ rimwe kumunsi) **. Ongeraho ** Tocilizumab ** (IL-6 yakira antagonist, $ 162 inyandiko {mg} $ buri munsi munsi)
** GCA NTA Bimenyetso BIGARAGARA (Kubabara umutwe, Ubwuzu bwo mu mutwe, cyangwa PMR + Ibimenyetso bya Cranial) ** ** Umuvuduko mwinshi wo mu kanwa Prednisone $ 40 inyandiko {kugeza} 60 inyandiko {mg} $ ($ 1 inyandiko {mg / kg / kumunsi} $, max $ 80 inyandiko {mg} $) rimwe mu gitondo ** yatangiye ako kanya muri ER cyangwa ku ivuriro. Ongeraho ** Tocilizumab ** (byagaragaye mu kimenyetso cyerekana * GiACTA * igeragezwa kugirango wikubye kabiri igipimo cyo kohereza kandi ugabanye cumulative steroid yose hamwe) cyangwa Methotrexate; amagufwa no kurinda igifu.
** Gusubiramo Biboneka Mugihe cya Steroid Taper ** Ako kanya usubire kuri ** IV Methylprednisolone $ 500 inyandiko {kugeza} 1.000 umwandiko {mg / umunsi} $ ** cyangwa kuzamuka ugaruka $ 60 inyandiko {kugeza} 80 inyandiko {mg / umunsi} $ umunwa prednisone + gutangiza ** Tocilizumab **. ** Iburira: ** Kuberako Tocilizumab ibuza IL-6, ** abarwayi bafata Tocilizumab bazagira CRP na ESR bisanzwe rwose ($ 0 inyandiko {kugeza} 1 inyandiko {mg / L} $) ndetse no mugihe cyo gusubiramo amashusho **; gukurikiranira hafi ibimenyetso byubuvuzi.

Tip

Niba utangiriye kuri prednisone ikabije ($ 40 inyandiko {kugeza} 80 inyandiko {mg} $ buri munsi) kuri Giant Cell Arteritis, ** ntuzigere uhagarika cyangwa ngo ugabanye urugero rwa prednisone mu buryo butunguranye wenyine **, nubwo umutwe wawe wabuze burundu nyuma yamasaha 48 cyangwa niba uhuye ningaruka mbi za steroid nko kudasinzira, jitterité, cyangwa isukari nyinshi yamaraso. Kubabara umutwe muri GCA mubisanzwe birashira mugihe cyamasaha 24 kugeza 48 yumuti wa mbere wa steroid, ariko gutwika arterial bisaba gukurikiranwa neza ** 12 kugeza 24-24 gahoro gahoro **. Guhagarika prednisone gitunguranye nyuma yicyumweru birashobora gukurura byombi ** guhumuka gutunguranye byombi ** no guhitana ubuzima ikibazo cya adrenal.

Inomero zihutirwa zo mukarere hamwe na protocole yikigo

  • ** Amerika na Kanada: ** Hamagara ** 911 ** kubera kubura amaso cyangwa ujye mu bitaro byegereye ishami ryihutirwa ryihutirwa hamwe n’indwara z’amaso. Kurikiza ** Ishuri Rikuru ry’Abanyamerika ryitwa Rheumatology (ACR) / Fondasiyo ya Vasculitis ** na ** Ishuri Rikuru ry’amaso ry’amerika (AAO) ** ritegeka amabwiriza ya methylprednisolone ya IV mbere ya biopsy yigihe gito.
  • ** Ubwongereza: ** Hamagara ** 999 ** (cyangwa ** 112 **) cyangwa witabe ishami ryihutirwa / ** NHS Kwitaho Amaso Yihutirwa / Ivuriro ryihuta rya GCA ** ako kanya. Munsi ya ** NICE ** na ** Sosiyete y'Ubwongereza ishinzwe indwara ya rubagimpande (BSR) ** Inzira yihuta ya GCA, abarwayi bafite ibimenyetso biboneka bahita bahura na methylprednisolone ya IV muri A&E hamwe na rheumatologiya yihutirwa / amaso y’amaso mu masaha 24.
  • ** Umuryango w’ubumwe bw’ibihugu by’i Burayi: ** Hamagara ** 112 **. ** EULAR (Umuryango w’ubumwe bw’ibihugu by’i Burayi bishinzwe indwara ya Rheumatologiya) ** amabwiriza arasaba guhita utangizwa cyane na glucocorticoid hamwe na ultrasound yihuta yigihe gito / axillary arteriire mu masaha 72 ku bigo byihariye by’imitsi.
  • ** Australiya na Nouvelle-Zélande: ** Hamagara ** 000 ** muri Ositaraliya cyangwa ** 111 ** muri Nouvelle-Zélande. Kurikiza ** Ishirahamwe rya Australiya na Nouvelle-Zélande ry’aba Neurologiste (ANZAN) ** na ** RANZCO (Royal Australiya na Nouvelle-Zélande College of Ophthalmologiste) ** protocole yihutirwa A-AION.
  • ** Ubuhinde na Aziya-Pasifika: ** Hamagara ** 112 ** mu Buhinde, ** 119 ** mu Buyapani na Koreya y'Epfo, cyangwa ** 995 ** muri Singapuru. Mu gihe GCA ifite umubare muto w’ibanze mu baturage ba Aziya ugereranije n’abaturage bo mu Burayi bw’Amajyaruguru, iracyagaragara buri gihe ku bantu bakuze barengeje imyaka 50 kandi ikunze kubura cyangwa gutinda; saba mu buryo bweruye ** statut ESR, CRP, hamwe nisuzumabumenyi ryinshi rya IV steroid ** kubantu bose bakuze bafite umutwe mushya, claudication, no kubura amaso.

Reba nimero yihutirwa kuri nimero mpuzamahanga zoherejwe.

Ibibazo bikunze kubazwa

Umuntu utarageza ku myaka 50 ashobora kurwara Arterite Yigihe gito (Arteritis Giant Cell)?

Indwara ya biopsy yemejwe na Arteritis igaragara cyane cyane kubantu bakuze bafite imyaka 50 nayirenga, aho abarwayi barenga 80 ku ijana barengeje imyaka 65 kubera ko imyaka ijyanye no guhindura imyaka ya arterial elastique lamina na senescence ikingira indwara. Ku murwayi utarageza ku myaka 40 cyangwa 50 ufite ibisa nini binini ndetse no gutakaza amaboko, abaganga basuzuma ** Takayasu Arteritis **, gutandukanya imiyoboro ya karoti, cyangwa izindi vasculitide sisitemu aho.

Niba narimaze gutakaza ijisho rimwe muri Giant Cell Arteritis uyumunsi, steroide yo mu mitsi izagarura amaso muri iryo jisho?

Iyo umutwe wa optique umaze kwandura indwara ya selile nini ya Arteritis, urupfu rwa fibre fibre ruhoraho rubaho vuba, bityo abarwayi bagera kuri 4 kugeza kuri 15% bonyine ni bo bafite uburwayi bwo kubona igice mumaso yamaze kwibasirwa ndetse na methylprednisolone ihita yinjira. Ariko, gutangira urugero rwinshi rwa IV steroid kurubu ni ibintu byihutirwa bihindura ubuzima kuko hatabayeho steroid ako kanya, abarwayi bagera kuri 50% bazabura amaso mumaso yabo ya kabiri (meza) muminsi 1 kugeza 7, bigatuma bahuma rwose mumaso yombi.

Kuki ntategereza iminsi 2 kugirango ntangire prednisone kugeza nyuma yo kubaga nyuma yo gukora biopsy yimitsi yigihe gito?

Gutegereza amasaha 24 kugeza 48 nta steroid kugirango biopsy "itavuwe" ni bibi kandi ntabwo ari ngombwa. Ubushakashatsi bwa Histopathologie bwerekana ko ibimenyetso byerekana ibimenyetso byinjira, ingirabuzimafatizo nini, hamwe no kurimbuka kwa lamina imbere bikomeza kugaragara neza imbere y'urukuta rw'imitsi y'agateganyo byibura iminsi 10 kugeza kuri 14 nyuma yo gutangira steroyide nyinshi, mu gihe buri saha imwe idafite steroyide ishobora guhuma amaso mu jisho ryawe.

Ni ukubera iki umuganga wanjye w'amenyo yasanze nta kibi kiri mu menyo yanjye igihe urwasaya rwanjye rubabaza igihe cyose nanyoye ibiryo?

Muri Arteritis ya Giant Cell, ububabare bwo mu rwasaya iyo uhekenye (** jaw claudication **) ntaho bihuriye namenyo yawe cyangwa urwasaya; biterwa na autoimmune inflammation igabanya imiyoboro ya maxillary na mumaso itanga amaraso akungahaye kuri ogisijeni mumitsi yawe yo guhekenya. Nkuko umurwayi ufite imitsi yamaguru yahagaritse kurwara inyana nyuma yo kugenda ibice bibiri, imitsi yawe yo guhekenya ibura ogisijeni nyuma yo guhekenya ibiryo amasegonda 60, kubabara kugeza igihe uhagaritse guhekenya, kandi ukaburira ibyago byugarije imitsi yijisho ryawe.

Nigute C-Reaction Protein (CRP) na ESR bipimisha amaraso nibisanzwe mugihe iyerekwa ryanjye ririmo kuba mubi mugihe mfata Tocilizumab (Actemra)?

Tocilizumab ikora ihagarika byimazeyo reseptor ya Interleukin-6 (IL-6), nicyo kimenyetso nyacyo cyimiti umwijima ukeneye gukora C-Reactive Protein (CRP) na fibrinogen (itwara ESR). Kubwibyo, umurwayi ufata Tocilizumab azahora afite CRP na ESR bisanzwe mubisanzwe mugupima amaraso kabone niyo Th1 yimitsi yimitsi yaba ikiri mumitsi yabo; abaganga bagomba gushingira ku bimenyetso by’amavuriro nko kubabara umutwe, kubabara mu rwasaya, cyangwa impinduka zigaragara aho kuba ibimenyetso byamaraso kugirango bamenye ko Tocilizumab yongeye kugaruka.

Disiki ya "chalky yera" optique isobanura iki mugihe umuganga wamaso areba mumaso yanjye?

Muburyo busanzwe, butari vasculitike yubwonko bwa optique (Non-Arteritic Anterior Ischemic Optic Neuropathie, cyangwa NAION), umutwe wibyimba wa optique wabyimbye usa nijimye cyangwa umutuku (hyperemic) ufite uduce duto twamaraso tumeze nk'umuriro. Iyo umuganga w'amaso abonye ** pallid, "chalky-yera" yabyimbye ya optic nervice ya disiki ** muminsi yambere yo kubura iyerekwa, byerekana infarction yuzuye yumuzunguruko wa ciliary kandi ni ikimenyetso cyiza cya ** Arteritike AION kuva muri Arterite nini ya Arteritis **.

Kuyobora

Inkomoko nubuyobozi

  • ** Ishuri Rikuru ry’Abanyamerika Rheumatology (ACR) na Fondasiyo ya Vasculitis (Amerika): ** * 2021 Ishuri Rikuru ry’Abanyamerika Rheumatology / Vasculitis Foundation Amabwiriza agenga imicungire y’imitsi nini ya Arteritis na Takayasu Arteritis * (Maz et al. kuvura).
  • ** Ihuriro ry’ibihugu by’i Burayi rishinzwe indwara ya rubagimpande (EULAR): ** * Ibyifuzo bya EULAR byo gucunga imiyoboro minini ya Vessel Vasculitis no gukoresha amashusho muri Vessel Vasculitis nini mu buvuzi bwa Clinical * (Hellmich et al.
  • ** Sosiyete y'Abongereza ishinzwe indwara ya rubagimpande (BSR, Ubwongereza): ** * Amabwiriza y’umuryango w’abongereza ushinzwe indwara ya rubagimpande ku gusuzuma no kuvura indwara ya Arterite nini * (Mackie et al., * Rheumatology *).
  • ** Ishuri Rikuru ry’amaso ry’Abanyamerika (AAO, Amerika) na Sosiyete yo muri Amerika y'Amajyaruguru ya Neuro-Ophthalmology (NANOS): ** * Amatangazo y’ubuvuzi yerekeye imicungire y’imicungire y’imitsi ya Arteritike Ischemic Optic Neuropathie (A-AION) no gukumira ubuhumyi bw'amaso *.
  • ** Royal College of Ophthalmologistes (RCOphth, Ubwongereza) na NICE: ** * Byihuta-Track Gigant Cell Arteritis Pathways hamwe nubuyobozi bwihutirwa bwo gutakaza ibintu bikabije *.

Note

Aka gatabo gatanga amakuru yihutirwa yubumenyi kandi ntisimbuza isuzuma ryubuvuzi bwumwuga cyangwa inzobere mu kuvura indwara ya neuro-ophthalmic na rheumatologic. Tekereza kwiga amasomo yambere yubufasha yemewe binyuze muri Croix-Rouge, Ambulance ya St John, cyangwa utanga amahugurwa yo muri ako gace kugirango umenye uburyo bwo kumenya ibyihutirwa byihuta, imitsi, n’ibyerekezo byihutirwa.