Giant Cell Arteritis (Temporal Arteritis): Disama dia tshiadi, Disama dia dikoba dia mutu, Diplopia, ne Diangatshila dia steroïdes dia bungi kumpala kua kuenza biopsie bua kusungila dîsu dibidi¶

Mu muntu mukulumpe yonso udi ne bidimu 50 anyi kupita apu, disama dia tshimpitshimpi dia mutu mupiamupia, disama dia mu mutu padibu bapanda nsuki, disama dia mu mishiku ya mu nshingu padiye utafuna biakudia (jaw claudication), dijimija dia tshimuenenu tshia tshitupa tshîpi, dimona dia misangu ibidi (diplopia), anyi difofoma dia tshimpitshimpi mu dîsu dimue bidi bualu bua lukasa bua luondapu budi bufumina ku ** selile ya mu tshiadi (GCA)**, udi kabidi mumanyike bu disama dia mishipa ya mu tshitupa tshîpi anyi disama dia Horton. Bikila nimero ya njiwu ya muaba unudi (911 mu États-Unis ne Canada, 112 mu Union européenne mujima, 999 mu Royaume-Uni) anyi yukila netu nomba ya tshimpitshimpi diakamue ne uya buludiludi ku tshibambalu tshia malu a mpukapuka tshia lupitadi bua stat high-dose intravenous corticosteroids. Patudi tulongolola mushindu wa kuendesha, enza dishilangana pankatshi pa dijimija dia dimona dia mu mishipa ne dia thromboembolique didi kadiyi dia mu mishipa disama dia tshiadi tshia mu mêsu (CRAO), tangila tshitupa tshietu tshinene dijimija dia tshimuenenu tshia tshimpitshimpi ne bulombodi bua ditapuluka dia retina, ne kutangila bimanyinu bia musangu umue bia buongo tshipupu.

Disama dia mishipa ya selile minene i disama dia mashi didi difumina ku selile ya T didi dikuata mishipa ya bunene bua munkatshi ne minene idi ne tshitupa tshia munda tshidi tshikolesha, ne dinanga dia patupu dia matamba a pambelu pa mutu a mishipa ya pambelu ne ya munda, nangananga mushipa wa pa mutu wa tshitupa tshîpi, ****, **mushipa wa opoph ne mishipa mikese ya panyima ya ciliaire. Padi disama dia intimal hyperplasia dikolesha bimanu bia mishipa mikese ya panyima too ne padi lumens yayi ijika, mutu wa muonji wa mêsu udi upeta disama dia tshipupu (arteritic anterior ischemic optic neuropathy [A-AION]), udi ukebesha bufofo bua bungi budi kabuyi mua kupingana (misangu ya bungi too ne padi muntu kayi umona butoke). Padi dîsu dia kumpala dijimija dimona, ditu dikola nansha padibu bamuondapa ne bukole. Kadi, kakuyi diondapangana dia lukasa dia bungi dia corticosteroid, babedi 30 too ne ku 50 pa lukama nebapete bufofo bua kashidi mu dîsu diabu dibidi (diabu) mu matuku 24 too ne matuku 7. Mikenji ya pa buloba bujima ya kudi American College of Rheumatology (ACR), EULAR, ne British Society for Rheumatology (RBS) idi ilomba ne: kufila methylprednisolone mu mushipa (500 too ne ku 1,000 milligrams ku dituku) anyi prednisone wa bungi diakamue paudi wela meji ne: udi ne disama, kuyi muindile mêba anyi matuku bua kupeta bipeta bia laboratware bia erythrocyte sedimentation rate (ESR), ultrasound wa mu mishipa ya tshitupa tshîpi, anyi biopsie ya mu mishipa ya tshitupa tshîpi.

Enza nunku mpindieu¶

1. Ndaku diakamue ku tshibambalu tshia lupitadi tshia lukasa lukasa (ki ntshia lupitadi lua lukasa lukasa, nzubu wa luondapu lua meno, anyi nzubu wa luondapu lua mêsu to) pikala muntu mukulumpe wa bidimu 50 anyi kupita apu upeta dishintuluka dia mêsu anyi bimanyinu bia disama dia tshiadi tshia mu mutu. Uondopa dimona dia tshitupa tshîpi didi kadiyi diumvuika bimpe (amaurose fugax), dimona dia kuulu anyi dia kuinshi didi kadiyiku (**dimona dibidi), dipanga dia dimona dia misangu ibidi (diplopia), anyi mutu mupiamupia ne disama dia tshiadi bu njiwu ya lukasa ya dimona dia ku nseke ibidi.
2. Ambila munganga ne munganga wa tshimpitshimpi mu tshiambilu tshieba tshia kumpala ne: "Tudi tuela meji ne: udi ne disama dia Giant Cell Arteritis didi ne njiwu ya kumona ne tudi dijinga ne manga a steroïde a bungi adibu bafila diakamue bua kusungila dîsu dibidi." Kuindidi anu mu nzubu wa kuindila wa mu lupitadi paudi ne dîsu didi kadiyi dimueneka bimpe to. Dîba dionso dia kulekela kumpala kua dose wa kumpala wa methylprednisolone (500 too ne ku 1000 mg IV) didi divudija njiwu ya se: tshipupu tshia disama tshidi mua kukanga mishipa ya kunyima ya dîsu dikuabu.
3. Kulekedi munganga ulengulula dibanga dia corticosteroïdes padiye muindile biopsie wa mushipa wa tshitupa tshîpi anyi ultrason wa mu lupitadi. Tshilema tshia munganga tshidi tshitamba kuenzeka ne tshidi tshinyangakaja ntshia kubenga kunua manga a prednisone anyi methylprednisolone bua buôwa bua se: manga a steroïdes ne "anyanga" tshipeta tshia biopsie wa mushipa wa tshitupa tshîpi. Disama dia granulomatose ne tuselile tunene tudi tushala tumueneka patoke mu tshimanu tshia mushipa wa tshitupa tshîpi munkatshi mua matuku 10 too ne ku 14 (ne misangu ya bungi too ne ku mbingu 4) kunyima kua dibanga dia manga a bungi a corticosteroïdes. Stéroïdes idi kumpala; biopsie udi ulua muibidi.
4. Umanye ne: "jaw claudication" ne "diplopia" bu bimanyinu bia didimuija dia kumpala kua kuikala mufofo. Bikala misunya ya mu mishiku yebe isama, mikole, anyi mikole kunyima kua minite 1 anyi 2 ya kudia nyama, mampa makole, anyi biakudia bikole ne udiumvua bimpe paudi ulekela kutafuna, etshi KI ntshilema tshia tshitupa tshîpi (TMJ) to. I disama dia ischémique dia masseter ne temporalis muscles didi difumina ku dijimija dia mishipa ya maxillary ne ya mpala, ne didi ne dipetangana dinene dia bungi ne bufofo budi pabuipi mu GCA. Bia muomumue, dimona dia misangu ibidi dia tshitupa tshîpi didi dilua kumpala kua bufofo bua kashidi mu mêba anyi matuku mu babedi batue ku 15 pa lukama.
5. Lala mubedi panshi (supine) pikala dijimija dia dimona dia lukasa anyi dijimija dia tshitupa tshîpi dimane kutuadija. Kulala panshi kudi kuvudija bukole bua mashi adi apita mu mishipa ya mêsu ne ya panyima idi ikolesha mutu wa muonji wa mêsu padibu balongolola methylprednisolone udibu bafila mu mushipa.
6. Tangila bimanyinu bia tshiadi tshia FAST ne bimanyinu bia vertebrobasilar. Bualu disama dia mishipa minene idi kabidi isama mishipa ya mu tshiadi ne mishipa ya munda mua tshiadi mu 3 too ne ku 7 pa lukama ya babedi, tangila ni udi ne disama dia mutu, dia mutu, dia dienda dibi, dia kuakula bibi, anyi dia butekete bua ku luseke lumue, ne manyisha diakamue malu aa kudi bena mudimu.

Mutshi wa dipangadika dia luondapu bua disama dia Giant Cell Arteritis kudi bantu bakole badi ne bidimu 50 ne ku mutu¶

Ingidija tshiamu etshi tshia disungula dia babedi bua kusungula babedi badi ne GCA mu malu a tshimpitshimpi a kumona/a ischémie ne GCA wa mu mutu udi kayi ne ischémie ne kutuadija diambuluisha dia lukasa dia steroïde.

flowchart TD
    Start["Bidimu bia muntu mukole >= Bidimu 50 bidi bileja ne tshintu tshionso:<br/>1. Mutu usama (Temple, Occipital, anyi General)<br/>2. Dikuma dia tshiadi anyi dia ludimi padibu batafuna<br/>3. Disama dia mu mutu anyi mushipa wa tshitupa tshîpi udi kayi ne nsongo<br/>4. Dipanga dia kumona dia tshimpitshimpi, Amaurosis Fugax, Diplopia, anyi Bufofo<br/>5. Luya ludi kaluyi lumvuija, dijimija dia kilo, anyi Polymyalgie Rheumatica"] --> VisualCheck{"Kudiku bimanyinu bia disama dia tshiadi tshia kumona anyi tshia ku mutu anyi?<br/>(Dijimija dia tshimuenenu, Amaurosis Fugax, Diplopia,<br/>anyi Disama dia Ludimi)"}

    VisualCheck -->|"EYOWA: MUSHINDU WA KUENZA / WA ISCHEMIE<br/>(Njiwu mikole ya bufofo bua ku nseke ibidi<br/>anyi AVC wa Vertebrobasilar)"| EmergencyIV["MUSHINDU WA KUENZA LUPILU:<br/>1. KUINDILA bipeta bia ESR/CRP anyi Biopsy<br/>2. Udi mua kufila Methylprednisolone wa bungi<br/> 500 too ne ku 1000 mg IV dituku dionso mu matuku 3 malondangane<br/> (Anyi Prednisone 60 too ne ku 80 mg PO Shi IV mupangile)<br/>3. Lala mubedi panshi (Supine) bua kupeta mashi a bungi<br/>4. Draw Stat ESR, CRP, CBC (Platelets), Fibrinogen"]

    EmergencyIV --> Maintenance["Panyima pa matuku 3 a IV Pulse Steroids:<br/>1. Kushintulula ku Prednisone 1 mg/kg/dituku<br/> (pa tshibidilu 60 mg ku dituku) + Weja Tocilizumab (IL-6 Blocker)<br/>2. Enza Ultrasound wa mushipa wa tshitupa tshîpi ('Halo Sign')<br/> ne Biopsie wa mushipa wa tshitupa tshîpi wa tshitupa tshîpi anyi wa tshitupa tshimue<br/> (> 1.5 too ne ku 2.0 cm mu bule) mu matuku 7 too ne ku 14<br/>3. CTA/MRA Aorta & Branches for Large-Vessel GCA"]

    VisualCheck -->|"NO: Mutu usama, disama dia mutu,<br/>anyi bimanyinu bia PMR ANYI<br/>(Dimona dia 100% diakane, Kakuyi dinyanguka dia tshiadi)"| NonIschemic["MUKANDA WA GCA WA DITUKU DIMUE:<br/>1. Funda Stat ESR, CRP, ne bungi bua mashi onso<br/>2. Tuanji kunua manga a bungi a ku mishiku diakamue:<br/> 40 too ne ku 60 mg PO dituku dionso (1 mg/kg/dituku) KUMPALA kua kuenza biopsie<br/>3. Ultrason wa lukasa lukasa wa mushipa wa tshitupa tshîpi<br/> ne biopsie wa mushipa wa tshitupa tshîpi mu mbingu 1 anyi 2<br/>4. Longesha mubedi bua kupingana ku ER diakamue pikala<br/> Disama dia tshimuenekelu, diplopia, anyi disama dia mu tshiadi dienzeke"]

Pathophysiology: Mushindu kayi udi auto-immune granulomatous vasculitis ushipa muonji wa mêsu¶

Kujingulula bua tshinyi disama dia mishipa minene didi dikuata mijilu kampanda ya bantu bakole badi ne bidimu bipite pa 50 kudi kumvuija bimanyinu biadi bia dikema (disama dia mu tshiadi, disama dia mu mutu, dikokangana dia maboko, ne difofo dia tshimpitshimpi) ne bua tshinyi manga adi ambuluisha bua kumbusha mashi adi afumina mu mishipa kaena mua kupingaja disama dia mijilu ya ku mêsu to.

1. Bua tshinyi disama dia Giant Cell Arteritis didi dikuata anu bantu bakole badi ne bidimu 50 ne ku mutu¶

Disama dia mishipa ya selile minene kadiena dienzeka nansha kakese kudi bantu badi ne bidimu muinshi mua 50**, ne bungi buadi budi buenda buvula bikole kunyima kua bidimu 50, difika ku tshibungubungu pankatshi pa bidimu 70 ne 80 (ne ditangalaka dia bungi mu bantu ba ku Mputu wa ku Nord ne ba mu Scandinavie, ne tshitupa tshia bakaji tshidi 2:1:1:1). - Elastic Lamina Senescence and Adventitial Dendritic Cells: Mishipa ya munkatshi ne minene idi ne lamina elastic wa munda (tshipese tshia mionji idi itapulula intima wa munda ne mishipa) ne idi idisha kudi mishipa mikese ya mashi idi mu tshimanu tshiabu tshia pambelu tshidibu babikila ne: vasa vasorum. Padi muntu upita pa bidimu 50, mashintuluka adi atangila bukulakaje mu mionji ya elastin ne dikola dia mubidi adi ambuluisha bua kuenza mudimu ne biamu bia pa buabi bidi bifuanangana ne toll pa adventitial dendritic cells bidi mu tshimanu tshia pambelu tshia mishipa ya mu mutu ne mu aorte thoracique. - T-Cell Macrophage Granulomatous Cascade: Activated dendritic cells recruit CD4+ T lymphocytes through vasa vasorum through two distinct inflammatory pathways: 1. Njila wa Interleukin-6 (IL-6) ne IL-17 (Th17), udi ulombola bintu bidi bienza mudimu mu tshifu (ESR, CRP, fibrinogen, ne thrombocytose udi wenda ubanda) ne udi ukebesha luya, tuishi tua butuku, dijimija dia bujitu, ne **hepatique (MR) ne 2. Interferon-gamma (IFN-\(\gamma\), Th1) ne macrophage pathway, udi usaka macrophages bua kudisangisha mu multinucleated giant cells udi upatula matrix metalloproteinases (udi unyanga tshitupa tshia munda tshia elastic lamina) ne platelet-derived growth factor (PDGF) ne vascular growth factor (GFVE). - Dikanga dia luminal ku diambuluisha dia hyperplasie wa munda (ki mashi a patupu to): Mu dikankamija dia PDGF, selile ya misunya milenga idi ibuela munda ne ivulangana lukasa, ifila hyperplasia wa munda munene. Tshitupa tshia munda tshia mushipa tshidi tshivula munda too ne padi tshitupa tshia munkatshi (lumen) tshifika too ne ku tshina anyi tshikanga 100 pa lukama.

+-------------------------------------------------------------------------+
|     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. Bua tshinyi dikanga dia mushipa wa kunyima didi difikisha ku disama dia "Chalky-White" Optic Nerve Infarction (A-AION)¶

Nansha mudi mushipa wa tshitupa tshia pa mutu udi pa mpala upeta dina dia disama edi, tshidi tshifikisha ku dipanga dia mêsu munda mua tshitupa tshia mpala tshidi pabuipi ne misangu yonso dibuikila dia mishipa mikese ya kunyima (SPCAs): - Pakatshi pa 10 ne 20 mishipa mikese ya panyima idi ipatuka mu mushipa wa mêsu ne itobola tshijengu (tshijengu tshia Zinn-Haller) buludiludi pabuipi ne mutu wa muonji wa mêsu (disque wa mêsu) bua kufila mutu wa muonji wa mêsu wa prelaminar ne wa laminar ne kabidi choroïde choroïde mujima. - Mu 80 too ne ku 85 pa lukama ya babedi badi ne dijimija dia dimona dia GCA, disama dia granulomatous vasculitis didi dikanga mijilu mikese ya panyima, difikisha ku Arteritic Anterior Ischemic Optic Neuropathy (A-AION). - Pa fundoscopy, mutu wa muonji wa mêsu udi munyanguke udi uvula ne ulua pathognomonic "chalky-white" (pallid) edema, mushilangane bikole ne edema wa pink/hyperemic udi umueneka mu benign non-arteritic AION anyi papilledema. - Mu 5 too ne ku 10 pa lukama, GCA udi ukanga mushipa wa tshiadi tshia munkatshi (Arteritic CRAO), ne mu 5 pa lukama udi ukanga mishipa ya retrobulbar posterior ciliaire kunyima kua globe (Posterior Ischemic Optic Neuropathy [PION], mudi mubedi muikale mpofu bikole ne tshilongelu tshia Marcus Marcus nansha mudiye umueneka bimpe menemene mu dituku dia kumpala).

Kujingulula misongo 6 minene ya disama dia Giant Cell Arteritis¶

Bu mudi disama dia mishipa ya selile minene mua kuikala bu disama dia mutu, disama dia menu/TMJ, disama dia sinus, arthrite, anyi disama dia tshimpitshimpi, baminganga ne babedi badi ne tshia kumanya bimanyinu bisambombo bia disama edi:

1. Disama dia tshiadi ne dia ludimi (Bimanyinu bia pa buabi)¶

• Dikoseka dia mushiku (didi mu 40% too ne ku 50% ya babedi ba GCA): Bishilangane ne disama dia tshiadi tshia temporomandibular (TMJ), didi difikisha muntu ku dikuma anyi kusama diakamue dîba didi muntu ukangula mukana muende munene anyi udima tshintu tshikole, dikosa dia mushiku didi difuanangane ne disama dia mutshima anyi disama dia tshiadi dia tshitupa tshîpi. Padi mubedi utuadija kutafuna biakudia bikole (bu mudi: nyama wa ngulube, nyama wa ngulube, bagels anyi mampa a kutafuna), bidia bikese bia kumpala kabitu bimusankisha to. Panyima pa sekonde 60 too ne ku 120 ya kutafuna misangu ne misangu, mishipa ya masseter ne ya temporalis idi ijika ne oxygène bualu mushipa wa maxillaire udi ne disama kena mua kuvudija dienda dia mashi to; ditshioka dikole, dia tshimpitshimpi, dia kanyinganyinga didi dikola mu nseke ya mpala, dienzeja muntu bua kulekela kutafuna ne kuikisha. Mu minite 1 anyi 2 ya dikisha dia mpala, disama didi dijika, anu bua kupingana diakamue padibu batuadija kutafuna. Mubedi udi ne GCA ne disama dia nshingu udi ne njiwu ya bungi ya misangu 3 too ne ku 4 ya bufofo bua kashidi kupita mubedi wa GCA udi kayi ne disama dia nshingu.
• Disama dia ludimi ne disama dia mu nshingu: Dipanga dia mashi mu mishipa ya ludimi ne ya mu tshiadi idi ibanda didi difikisha ku disama dia ludimi anyi dipanga dia lungenyi padi muntu wakula anyi udia, disama dia ludimi padibu bamina (odynophagia), anyi disama dia ludimi didi dinyanguka/ditoka didi mua kulua gangrene/nekrose wa ludimi pikalabu kabayi bamanye bibi.

2. Mutu usama, dikoba dia mutu, ne mishipa ya mu tshitupa tshîpi idi kayiyi mimpe¶

• Mutu mupiamupia (udiku mu 70% too ne ku 85%): Mushindu mupiamupia wonso wa mutu udi usama mu muntu mukole udi ne bidimu bipite pa 50 (anyi dishintuluka dinene dia mushindu udi muntu udi ne disama dia mutu usama mu matuku ende onso a muoyo) udi tshimanyinu tshikunze. Nansha mudibi biumvuika misangu yonso, bidi bitonda anyi bikolesha pa mutu pa tshimue anyi bionso bibidi, mutu wa GCA udi mua kuikala wa kumpala, wa kunyima (kunyima kua tshifu ku diambuluisha dia disama dia mushipa wa tshiadi), anyi wa tshimpitshimpi.
• Disama dia mu mutu (Allodynia): Disama dia matamba a mu mishipa ya pa mutu ne ya kunyima idi ifila dikoba dia mutu didi difikisha ku disama dia bungi padibu bapanda anyi basukula nsuki, bowa nsuki mu tshina tshia mâyi, bavuala manyanu a mêsu anyi tshilamba, anyi balala mutu pa tshilamba tshia kulala butuku. Mu bilumbu bikole bidi kabiyi biondapibua, bilonda bia mubidi bidi bisama (necrose wa ku mutu) bidi bimueneka pa mutu pa mutu wa kumpala ne bidi mua kuikala biumvuika bu bilonda bia shingles (herpes zoster).
• Dikonkonona dia mubidi dia mushipa wa tshitupa tshia pa mutu: Kulenga bitekete bitekete kumpala kua ditshi (tragus) ne ku luseke lua ntempelo kudi mua kuleja mushipa wa tshitupa tshia pa mutu udi umvua mukole, muikale ne nsongo ("muenza ne mabue"), mukole bu tshitupa tshia nkamba anyi tshingoma, mupepele bua kulenga, mutoke, ne mutekete anyi kayiku.

3. Biyukeno bya kumona: Amaurosis fugax, diplopia, ne A-AION¶

• Dijimija dia tshimuenenu tshia tshitupa tshîpi (Amaurosis Fugax mu 20% too ne ku 30%): Matuku anyi mêba kumpala kua bufofo bua kashidi, bantu batue ku tshia bisatu tshia babedi batu bapeta bikondo bikese bia minite 1 too ne ku 15 mudi dimona dia mu dîsu dimue dimueneka bu dia lupuishi, dinyanguka bu foto mutoke bikole, anyi dibuikila kudi mîdima, imue misangu kuimana pa nkuasa (postural optic nerve ischémie bualu mashi adi apitshila ku tshimanu tshia mishipa).
• Diplopie wa tshitupa tshîpi anyi wa kashidi (Dimona dia misangu ibidi mu 6% too ne ku 15%): Padi GCA usama mijilu mikese idi ifila biakudia ku misunyi ya pambelu pa mêsu anyi mijilu isatu, inayi anyi isambombo ya mu mutu munda mua orbite, mêsu abidi adi ajimija bulongame, ne adi afikisha ku dijimina dia tshimpitshimpi dia **dimona dia muulu anyi dia muulu (didi dibuikila kabidi dîsu). Mu muntu mukole udi ne bidimu bipite pa 50 udi ne mutu usama anyi usama mu nshingu, diplopie wa tshitupa tshîpi udi tshipupu tshia kumpala kua bufofo tshidi ne alarme itanu.
• Difofo dia kashidi dia muulu anyi dia tshishiki: Babedi batu misangu ya bungi bajuka mu dinda anyi bamona mu tshimpitshimpi mu munya ne: tshitupa tshijima tshia kuinshi anyi tshia kuulu tshia dimona dia dîsu dimue tshidi tshifiike (tshilema tshia dimona dia muulu), anyi ne: dîsu dijima ndilue mîdima too ne ku dibala dia minu anyi ** ** butoke bua kumona) (NLP**).

4. Polymyalgia Rheumatica (PMR) ne bimanyinu bia B bia mubidi mujima¶

• Polymyalgie Rheumatica (Mu 40% too ne ku 50% ya babedi ba GCA): Kadi, pabuipi ne 15 to ne 20% ya babedi badibu bapeta ne PMR badi ne GCA udi kayi umueneka anyi udi ulonda. PMR udi ukebesha disama dikole dia ku nseke ibidi ne dikolesha dia mu dinda didi dienza minite mipite pa 45 too ne ku 60 mu mifuba ya pa makaya, mu nshingu, ne mu mikaba ya mu tshiadi/mu tshiadi, bienza bua bikole bua mubedi kubandisha bianza biende pa mutu pa makaya ende bua kuvuala anyi kuimana mu nkuasa mupuekele kayi wenza mudimu ne bianza biende.
• Occult "Silent" Systemic GCA (Fever of Unknown Origin): Bantu batue ku 10 too ne ku 15 pa lukama badi ne disama dia GCA badi ne mutu usama anyi disama dia mutu ne badi baleja mbingu ya kapia ka dituku dionso kadiyi diumvuija, tuishi tua butuku tudi tuela mâyi, anorexie mukole, kilo 15 too ne ku 11 to (21) to kilo) ya dijimija dia bujitu dia tshianana, ne disama dia normocytic anemie, anyi badi baleja bufofo bua tshimpitshimpi budi kabuyi ne kanyinganyinga mu dîsu dimue budi buenda anu ne ESR ne CRP bidi bikola.

5. Bilumbu bia mishipa minene ne ya mu buongo (AVC ne dikosolola dia aorte)¶

• Vertebrobasilar ne Carotid Ischemic Stroke (3% to 7%): GCA mmunange bitupa bia pambelu pa vertebral arteries mudibi bibuela mu dura (bualu lamina elastic mmukole kuntu aku). Misangu yonso ikala utangila bimanyinu bia disama dia tshiadi tshia panyima (tshitupa tshia mutwe, ataxie, dysarthrie, dysphagie, hemianopie homonymous).
• Large-Vessel Subclavian/Axillary Stenosis and Thoracic Aortic Aneurysm and Thoracic Aortic Aneurysm: Mu bantu 25 too ne ku 50 pa lukama badi ne disama dia GCA, tshimfuanyi tshia PET-CT anyi CTA tshidi tshileja disama dia aorta wa mu tshiadi, dia mishipa ya muinshi mua dikasa, ne dia mishipa ya mu tshiadi, didi difikisha ku dikuma dia **mutoyi padi mashi afika ku mutu, ntatu idi pankatshi pa diboko dia balume ne dia balume (> 10 too ne ku 15 mmHg dishilangana), dijimija dia tshiadi tshia radial, anyi dipanga dia aneurysme thoracique aortique et dissection.

Dishilangana dia AION wa mu mishipa (GCA) ne AION udi kayi wa mu mishipa (NAION)¶

Bualu Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION) udi mu bulelela kupita Giant Cell Arteritis mu bantu bakulumpe ba bidimu bia pankatshi ne bakulumpe (udi ufumina ku dipueka dia mashi mu butuku ne ku dibuela dia mijilu mikese mu tshitupa tshikese tshia mêsu pamutu pa disama dia mijilu), baminganga ba mêsu ne baminganga ba malu a mpukapuka batu benza mudimu ne disama edi bua kuambuluisha Tapulula bintu bibidi ebi diakamue:

Mudimu wa dikonkonona dia masama ne bulongolodi bua luondapu lua tshibambalu tshia malu a mpukapuka¶

+-------------------------------------------------------------------------+
|     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. |
+-------------------------------------------------------------------------+

Bua tshinyi bule bua tshitupa tshia mashi (\(\ge 1.5\text{ to }2.0\text{ cm}\)) budi ne mushinga: Tshilumbu tshia "Skip Lesion"¶

Disama dia mishipa ya selile minene kadiena dikolesha bule buonso bua mushipa mu mushindu umue to; pamutu pa nanku, udi uluisha mu bitupa bidi kabiyi bilondangana bidibu babikila ne: "skip lesions," mudi tshitupa tshia 5-millimetre tshia mushipa udi ne disama dikole ne selile minene ya multinucleated ne tshitupa tshia elastic lamina tshidi tshitambakane tshisombe anu ku luseke lua tshitupa tshia 5-millimetre tshia tshimanu tshia mushipa tshidi tshimpe menemene. - Nunku, padi munganga wa mijilu, munganga wa mêsu, anyi munganga wa mishiku wenza biopsie wa mishipa ya tshitupa tshîpi (tshienzedi tshia minite 30 tshidi katshiyi ne njiwu muinshi mua anesthesie wa muaba udibu basombele ku luseke lua nsuki ku luseke lua tshimanyinu), mikenji idi ilomba bua kuangata tshitupa tshia mishipa tshidi ne bule bua **1.5.500 too ne ku ** ** ** (panyima pa dienza dia liline). shrinkage) ne kukonkonona bipese bia bungi bia mulongolongo bua bilonda bia skip kabiyi bipua muoyo. - Nansha shi biopsie wa mushipa wa tshitupa tshîpi mupingane bibi (udi wenzeka mu 10 too ne ku 15 pa lukama ya bilumbu bia GCA bia bushuwa bua bilonda bia skip anyi bua dibuela dia extracranial/posterior ciliary), biopsie mubi kena muanji kupita tshijadiki tshia kale tshia ESR/CRP ne dijimija dia mêsu; baminganga badi batungunuka ne luondapu lua corticosteroid ne tocilizumab.

Ultrason wa lukasa ("Tshimanyinu tshia Halo") ne MRI wa resolution wa bungi¶

Bibambalu bia bungi bia matuku etu aa bia diondopangana dia mikolo ne bia njiwu bidi mpindieu bienza ultrasonographie duplex de couleur rapide ya mishipa ya pa mutu (ya kumpala ne ya ku mutu) ne ya mu tshiadi mu mêba 24 too ne ku 48 a dimanyisha: - Disama dia granulomatous dia mu tshimanu tshia mashi didi dipatula tshijengu tshifiike (hypoechoic), tshidi tshinyunguluka tshijengu tshia Doppler tshia dikala dia mashi tshidibu babikila ne: "Halo Sign," tshidi katshiyi tshifika padi tshiamu tshia ultrason tshifina pa dikoba (positive "Compression Sign"). - Bilondeshile EULAR ne ACR guidelines, tshimanyishilu tshimpe tshia tshiamu tshia ultrasound tshia tshikondo/tshia tshiadi mu mubedi udi ne mushindu wa bungi wa kuondopa tshidi tshijadika disama dia GCA ne bujalame bua bungi, eku 3-Tesla cranial vessel-wall MRI wa resolution wa bungi anyi FDG-PET/CT wa mubidi mujima mua kuleja disama dia mishipa minene ne subclavitorite.

Mukanda wa diyukidilangana mulelela bua disungula dia mu lupitadi¶

Paudi ufika mu tshibambalu tshia malu a mpukapuka mu lupitadi ne muntu mukulumpe udi ne bimanyinu bia kumona anyi bimanyinu bia mu mutu bia disama dia mishipa minene, bala mukanda eu buludiludi kudi munganga wa malu a mpukapuka ne munganga wa malu a mpukapuka:

"Ndi apa ne mubedi wa bidimu [Age] uvua mupete [dijimija dia tshimuenenu / dijimija dia tshimuenenu tshia tshitupa tshîpi / dimona dia misangu ibidi] mu dîsu dia [dia bakaji/dia balume] lelu, kumpala kua [mutu mupiamupia wa ku ntempelo / disama dia ku mutu padibu basukula nsuki / disama dia mu nshingu panyima pa ditafuna biakudia munkatshi mua minite umue]. Tudi tuela meji bikole ne: Giant Cmpoterial Arteris (Temperitis acute) udi ne Arteritis Arteristique wa tshimpitshimpi. njiwu ya disama dia mitu ya ku mêsu. Bualu 30 too ne ku 50 pa lukama ya babedi badi kabayi baondapibua batu bafua mu disu diabu dibidi mu matuku makese, mikenji ya ACR ne ya EULAR idi ilomba bua kuela methylprednisolone (500 too ne ku 1000 mg IV) diakamue mu nzubu wa luondapu lua lukasa kumpala kua kuindila bipeta bia CRP anyi bia tshitupa tshîpi bia mashi. biopsie. Suaku lomba munganga wa tshimpitshimpi bua kukonkonona mubedi diakamue."

Mulongo wa bantu badi ne disama dia Polymyalgia Rheumatica (PMR) anyi steroids¶

Shi wewa nansha muntu mukulumpe wa mu kisaka ukadi mupeta disama dia Polymyalgia Rheumatica (PMR) anyi udi mpindieu wenda ukepesha manga a prednisone bua Giant Cell Arteritis, ela liste eu mu frigo webe ne upingane mu nzubu wa lupitadi diakamue pikala bualu kampanda buenzeke:

• Dijimina dia tshitupa tshîpi, dipanga dia mpala, lupuishi, anyi rido pa dîsu dimue (nansha biobi bijika kunyima kua minite 2).
• Tshitupa tshionso tshia dimona dia bintu bibidi (kumona bimfuanyi bibidi ku luseke ne ku luseke anyi pa mutu pa tshikuabu), nansha biobi bia tshitupa tshîpi.
• Ditshioka dipiadipia, disama, anyi disama dia mu tshiadi anyi dia ludimi padibu batafuna biakudia.
• Dipingana dia mutu usama anyi dibungama padibu batenga dikoba dia mutu anyi padibu bateka makalashi.
• Kulekedi anyi kupuekesha manga a prednisone anyi tocilizumab mu tshimpitshimpi to: Kulekela manga a steroïdes mu tshimpitshimpi mu tshikondo tshia GCA udi wenza mudimu kudi mua kufikisha ku dipingana dia bufofo ne disama dia adrenal acute didi mua kunyanga muoyo (tangila bulombodi bua tshimpitshimpi tshia disama dia tshiadi).

Nkonko itubu bela misangu ya bungi¶

Bua tshinyi baminganga batu bafila manga a bungi a steroïde mu mishipa bua Giant Cell Arteritis kumpala kua kuenza biopsie wa mu mishipa ya tshitupa tshîpi?¶

Mu disama dia Giant Cell Arteritis, padi mashi adi aya ku muonji wa mêsu ajika mu dîsu dia kumpala, dimona dia mu dîsu adi ditu anu dijimina kashidi, ne padibu kabayi babaondapa diakamue ne manga a steroïde, babedi 30 anyi 50 pa lukama nebajimije dimona dia dîsu diabu dimpe didi dishala mu mêba 24 too ne matuku 7. Kutuadija difila dia methylprednisolone mu mishipa kudi kujika diakamue disama dia mu mishipa bua kusungila dîsu dibidi, ne bamanyi ba malu a masama badi anu mua kumanya patoke disama dia mu tshimanu tshia mishipa pa biopsie wa mushipa wa tshitupa tshîpi muenza too ne mbingu 2 kunyima kua dibanga dia steroïdes.

Mmunyi mundi mua kumanya dishilangana pankatshi pa disama dia tshiadi tshia Giant Cell Arteritis ne disama dia tshiadi dia TMJ anyi disama dia meno?¶

Disama dia temporomandibular joint (TMJ) ne disama dia meno bidi bifila kanyinganyinga kakole diakamue dîba diudi uluma bikole, ukangula mukana muebe bikole, anyi ututa pa dinu kampanda, misangu ya bungi didi dilondangana ne disama dia tshiadi didi dikuma. Disama dia tshiadi didi difumina ku Giant Cell Arteritis didi lutatu lua difila dia mashi mu mishipa anu bu mudi disama dia mutshima: bidia biebe bia kumpala bidi biumvuika bimpe, kadi kunyima kua minite 1 anyi 2 ya ditafuna dia tshiendelele (nangananga nyama anyi mampa makole), mishipa yebe ya mu tshiadi idi ipeta kanyinganyinga kakole ne ditshioka didi dikuenzeja bua kulekela kutafuna, kanyinganyinga ne kujika panyima pa minite 1 to dikisha.

Muntu udi ne bidimu muinshi mua 50 udi mua kupeta disama dia Giant Cell Arteritis (Temporal Arteritis) anyi?¶

Disama dia Giant Cell Arteritis didi dienzeka anu kudi bantu bakole badi ne bidimu 50 ne ku mutu (badi ne bidimu 72 too ne ku 76) bualu tshienzedi tshia auto-immune tshidi tshijudibua kudi mashintuluka adi atangila bidimu mu bitupa bia mishipa ya munkatshi ne minene. Padi nsonga udi ne bidimu muinshi mua 40 upeta disama dia mishipa minene ya mu mishipa ya mu mikolo ne ya mu mishipa ya mu mikolo anyi ya mu tshiadi tshia muinshi mua tshiadi, didi disama didi dipetangana kadi dishilangane didibu babikila ne: Takayasu arteritis ("disama didi kadiyi ne tshiadi").

Biopsie wa tshitupa tshîpi udi kayi muimpe udi umvuija tshinyi pikala munganga wanyi utshidi wela meji ne ndi ne disama dia Giant Cell Arteritis?¶

Bualu Giant Cell Arteritis udi uluisha mishipa mu "skip lesions" (udi ushiya mishipa mimpe pankatshi pa mishipa idi isama) anyi udi mua kuenza bua mishipa ya ciliaire ya panyima pa dîsu ikale ne disama dia panyima pa dîsu eku kayi ulama ditamba dia mpala, 10 too ne ku 15 pa lukama ya babedi badi bushuwa ne Giant Cell Arteritis nebapete biopsie biakane. Nunku, shi bimanyinu biebe, bidimu biebe, ne mateta a mashi a ESR/CRP anyi "tshimanyinu tshia halo" tshia ultrasons bileja bikole ne: udi ne disama dia Giant Cell Arteritis, baminganga nebatungunuke ne kukuondopa ne steroïde bua kukuba dimona diebe nansha mudi biopsie mubi.

Miaba mifumina¶

• American College of Rheumatology (ACR) and Vasculitis Foundation (United States): 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis (Maz et al., *Arthritis & Rheumatology, recommending high-immediate intravense glucocorticoids [methylprednisolone 500 to 1,000 mg/day] bua GCA ne dijimija dia mêsu anyi amaurosis fugax kumpala kua biopsie, pamue ne dikumbaja dia tocilizumab).
• European Alliance of Associations for Rheumatology (EULAR): EULAR Recommendations for the Management of Large Vessel Vasculitis and EULAR Recommendations for the Use of Imaging in Large Vessel Vasculitis in Clinical Practice (fast-track temporal steillax/media "sounds," ntuadijilu, ne \(\ge 1.5\text{ cm}\) bule bua biopsy wa panyima pa dijadika).
• British Society for Rheumatology (BSR) and Royal College of Ophthalmologists (United Kingdom): British Society for Rheumatology Guideline on Diagnosis and Treatment of Giant Cell Arteritis (Mackie et al., emergency intravenous methylprednisolone pathways and difference of A-ION vs. NA.AION).
• New England Journal of Medicine (GiACTA Trial): Trial of Tocilizumab in Giant-Cell Arteritis (Stone et al., showing sustained glucocorticoid-free remission with a weekly subcutaneous receptor receptor IL-6).

Malu adi apetangana¶

• Dijimija dia tshimuenenu tshia tshimpitshimpi, ditapuluka dia retina, ne glaucome wa angle-closure wa tshimpitshimpi
• Dibuikila dia mushipa wa tshiadi tshia munkatshi (CRAO) tshipupu tshia mêsu ne dididishi dia minite 90
• Glaucome wa angle-closure wa tshimpitshimpi: tshimuenenu tshia halo, dîsu dikole bu dibue, ne bua tshinyi nzubu ya mîdima idi inyanga dikanga dia mêsu
• Dijingulula dia tshipupu (FAST) ne diandamuna dia tshimpitshimpi
• Disama dia adrenal ne disama dia Addison ditua dia steroïde mu tshimpitshimpi