Antiphospholipid syndrome - APS

Antiphospholipid syndrome (APS) o se faʻamaʻi autoimmune e aofia ai le tele o faʻamalosi toto (thromboses).A maua ia oe le tulaga lea, o le tino puipuia o lou tino e faia ai proteins le masani ai e osofaʻia le toto sela ma le ufiufi o alatoto toto. O le iai o nei vailaʻau e mafai ona mafua ai faʻafitauli ma le tafe o le toto ma mafua ai le afaina o le toto i ala uma o le tino.

Ole mafuaʻaga tonu ole APS e leʻo iloa. O ituaiga gafa e lua ma isi mea taua (pei ole faʻamaʻi) e ono tupu ai le faʻafitauli.

E masani ona maua i tagata e iai isi faʻamaʻi ole autoimmune, pei ole systemic lupus erythematosus (SLE). O le tulaga e sili atu taatele fafine nai lo alii, E masani ona maua i fafine o loʻo i ai le talaʻaga o le faʻatele o maʻitaga.

Nisi tagata o loʻo aveina ni vailaʻau na taʻua i luga, ae leai ni APS. O nisi mafuaʻaga ono mafua ai nei tagata ia maua se toto toto, aofia ai:

• Ulaula
• Moega umi moega
• Maitaga
• Hormone togafitiga poʻo fualaʻau taofiofia fana
• Kanesa
• Faamai fatugao

Oe ono leai ni faʻailoga, e ui lava o loʻo ia te oe ni vailaʻau. Faʻailoga e ono tupu e aofia ai:

• Toto toto i vae, lima poʻo le mama. O toʻotoʻo e mafai ona i totonu o uaua poʻo alatoto.
• Faʻafuaseʻi o maʻitaga poʻo fanau pea.
• Rash, i nisi tagata.

I tulaga seasea tupu, faʻafuaseʻi lava ona tupu ai ni toʻafu i le tele o alatoto i se vaitaimi o aso. E taʻua lea mea o le catastrophic anti-phospholipid syndrome (CAPS). E mafai ona taitai atu ai i le afaina faapea foi ma le gagala i le fatugaʻo, ate, ma isi totoga i le tino atoa, ma gangrene i vae ma lima.

Suʻega mo le lupus anticoagulant ma antiphospholipid vailaʻau mafai ona faia pe a:

• O le toto e leʻi faʻamoemoeina e tupu, e pei o talavou pe i latou e leai ni isi tulaga lamatia mo le toto toto.
• O se fafine ua i ai se talaʻaga o le toʻatele maʻitaga maʻimau.

O suʻesuʻega ole lupus anticoagulant o le tui toto. O le antiphospholipid antibodies (aPL) mafua ai le suʻega e le masani ai i le fale suesue.

Ituaiga o tui faʻataʻitaʻiga ono aofia ai:

• Faʻagaoioi vaega thromboplastin taimi (aPTT)
• Le taimi o leona feʻai a Russell
• Suʻesuʻega taofiofi ole thromboplastin

Suʻega mo vailaʻau faʻasaʻi antiphospholipid (aPL) o le a faia foi. E aofia ai:

• Anticardiolipin antibody tofotofoga
• Antibodies i le beta-2-glypoprotein I (Beta2-GPI)

O lau fomaʻi tausi soifua o le a faʻamaonia le antiphospholipid antibody syndrome (APS) pe a fai o iai sau suʻega lelei mo le APL poʻo le lupus anticoagulant, ma le tasi poʻo le sili atu o mea nei.

• O se toto masaa
• Faʻamamona faʻalua

O faʻataʻitaʻiga lelei e manaʻomia e faʻamaonia pe a maeʻa le 12 vaiaso. Afai e i ai sau suʻega lelei e aunoa ma isi foliga o le faʻamaʻi, oe le maua le faʻamaoniga o le APS.

O le togafitiga mo le APS e faʻatonutonuina i le puipuia o faʻalavelave mai le toto fou ua maua pe o loʻo iai ni faʻaputuga o loʻo faʻateleina. Oe e manaʻomia le inuina o nisi ituaiga o toto-manifinifi vailaʻau. A iai sau faʻamaʻi ole autoimmune, pei ole lupus, e manaʻomia foi ona e taofiofia lena tulaga.

O le togafitiga saʻo e faʻamoemoe i le ogaoga o lou tulaga ma faʻalavelave o loʻo mafua ai.

ANTIPHOSPHOLIPID ANTIBODY Syndrome (APS)

I se tulaga lautele, oe o le a manaʻomia togafitiga ma se toto manifinifi mo se taimi umi pe a fai oe APS. Muamua togafitiga ono heparin. O nei vailaʻau e avatua i le tui.

I le tele o tulaga, warfarin (Coumadin), lea e tuʻuina e le gutu, ona amataina lea. E manaʻomia le mataʻituina masani o le tulaga ole anticoagulation. E masani ona faia lenei mea ile faʻaaogaina ole INR suʻega.

A iai sau APS ma maʻito, e manaʻomia ona mulimulitaʻi oe se tautua o loʻo atamamai i lenei tulaga. Oe o le a le avea warfarin i le taimi o maitaga, ae o le a tuʻuina atu heparin tui nai lo.

Afai e iai sau SLE ma le APS, o le a fautuaina foʻi oe e lau tautua e te inuina le hydroxychloroquine.

I le taimi nei, o isi ituaiga o vailaʻau faʻamaligiina o le toto e le fautuaina.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME (CAPS)

Togafitiga mo CAPS e aofia ai le tuʻufaʻatasia o anticoagulation togafitiga, maualuga tui o corticosteroids, ma fesuiaʻiga plasma sa aoga i le tele o tagata. O isi taimi e faʻaaogaina ai le IVIG, rituximab poʻo le eculizumab mo ni matuiā.

POSITIVE TEST FOR LUPUS ANTICOAGULANT PO O APL

E te le manaʻomia se togafiti pe a fai e leai sau faʻailoga, maʻito toʻesea, pe afai e te leʻi maua lava i se toto toto.

Ia uia laʻasaga ia e fesoasoani e puipuia ai le faʻapipiʻi o le toto.

• Aloʻese mai le tele o fualaʻau poʻo togafitiga o homone mo menopause (fafine).
• AUA le ulaula pe faʻaaoga isi tapaa.
• Tu i luga ma fealualuaʻi i taimi uumi vaalele vaalele po o isi taimi pe a e tatau ona nofo pe taoto i lalo mo vaitaimi uumi.
• Faʻasolosolo ou tapuvae i luga ma lalo pe a le mafai ona e fealuaʻi.

O le a faʻatonuina oe e faʻavaivaia le toto (e pei o le heparin ma le warfarin) e fesoasoani e puipuia ai le faʻaputuga o le toto:

• Ina ua uma taotoga
• Ina ua maeʻa le ponaivi gaui
• Ma le kanesa malosi
• A e manaʻomia le nofo pe taoto i lalo mo se taimi umi, pei o le taimi o le falemaʻi nofo pe toe faʻaleleia i le fale

Atonu e manaʻomia foʻi ona e inu le faʻamaeʻa toto mo le 3 i le 4 vaiaso peʻa maeʻa le taotoga e tuʻu ai i lalo le aʻafiaga o le toto.

A aunoa ma togafitiga, tagata ma APS o le a toe faia faʻaputuputuina. O le tele o taimi, o le iʻuga e lelei ma le tatau ai o togafitiga, e aofia ai ma togafitiga faʻafomaʻi mo se taimi umi. O nisi tagata e mafai ona maua i le toto papala e faigata ona taofiofia e ui i togafitiga. Lenei mafai ona taitai atu i CAPS, lea e mafai ona lamatia ai le ola.

Valaʻau lau tautua pe a e maitauina faʻailoga o le toto toto, pei o:

• Fula pe mumu i le vae
• Manava le mānava
• Tiga, numbness, ma sesega paʻu lanu i se lima poʻo se vae

Talanoa foʻi i lau tautua pe a fai o oe ua tele le maʻitaga (maitaga).

Anticardiolipin antodies; Hughes syndrome

• O le systemic lupus erythematosus mageso i foliga
• Toto toto

Amigo M-C, Khamashta MA. Antiphospholipid syndrome: pathogenesis, faʻamaoniga, ma pulega. I: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Filatelefia, PA: Elsevier; 2019: mata 148

Cervera R, Rodríguez-Pintó I, Colafrancesco S, et al. Lipoti a le Vaega Faʻavaomalo Faʻavaomalo i le Antiphospholipid Antibodies Task Force e uiga i le catastrophic antiphospholipid syndrome. Autoimmun Rev. 2014; 13 (7): 699-707. PMID: 24657970 www.ncbi.nlm.nih.gov/pubmed/24657970.

Dufrost V, Risse J, Wahl D, Zuily S. Direct oral anticoagulants faʻaaogaina i le antiphospholipid syndrome: o nei vailaʻau o se aoga ma sefe auala e sui ai warfarin? O se iloiloga faʻavasega o tusitusiga: tali i manatu. Curr Rheumatol Rep. 2017; 19 (8): 52. PMID: 28741234 www.ncbi.nlm.nih.gov/pubmed/28741234.

Erkan D, Salmon JE, Lockshin MD. Anti-phospholipid syndrome. I totonu: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley ma Firestein's Tusitusiga o Rheumatology. 10 ed. Filatelefia, PA: Elsevier; 2017: mata 82.

National Heart, Lung, ma le Blood Institute 'upega tafaʻilagi. Antiphospholipid antibody syndrome. www.nhlbi.nih.gov/health-topics/antiphospholipid-antibody-syndrome. Avanoa Iuni 5, 2019.