Isifo se-Antiphospholipid - APS

I-Antiphospholipid syndrome (i-APS) yisifo esizimele esihilela amahlule egazi (thromboses).Uma unalesi simo, amasosha omzimba wakho enza amaprotheni angajwayelekile ahlasela amangqamuzana egazi nolwelwesi lwemithambo yegazi. Ukuba khona kwala ma-antibody kungadala izinkinga ngokugeleza kwegazi futhi kuholele kumahlule ayingozi emithanjeni yegazi emzimbeni wonke.

Imbangela ngqo ye-APS ayaziwa. Kokubili izinguquko ezithile zofuzo nezinye izinto (njengokutheleleka) kungadala inkinga.

Kuvame ukutholakala kubantu abanezinye izifo ezizimele, njenge-systemic lupus erythematosus (SLE). Isimo singabantu besifazane abavame kakhulu kunabesilisa, Sivame ukutholakala kwabesifazane abanomlando wokukhulelwa kwezisu okuphindaphindwayo.

Abanye abantu baphatha ama-antibody ashiwo ngenhla, kepha abanayo i-APS. Izimbangela ezithile zingadala ukuthi laba bantu babe negazi, kufaka phakathi:

• Ukubhema
• Ukuphumula kombhede isikhathi eside
• Ukukhulelwa
• Ukwelashwa kweHormone noma amaphilisi okulawula ukubeletha
• Umdlavuza
• Isifo sezinso

Ungahle ungabi nazimpawu, noma ngabe unamasosha omzimba. Izimpawu ezingenzeka zifaka:

• Amahlule egazi emilenzeni, ezingalweni noma emaphashini. Amahlule angaba semithanjeni noma emithanjeni yegazi.
• Ukuphuphuma kwesisu okuphindaphindiwe noma ukuzalwa kusenzekile.
• Rash, kwabanye abantu.

Ezimweni ezingavamile, amahlule avele ngokuzumayo emithanjeni eminingi esikhathini esiyizinsuku. Lokhu kubizwa nge-anti-phospholipid syndrome (CAPS) eyinhlekelele. Kungaholela ekushayweni unhlangothi kanye nokuqina kwamazinyo ezinso, isibindi, nezinye izitho emzimbeni wonke, kanye nokubola kwezitho zomzimba.

Ukuhlolwa kwama-antibody e-lupus anticoagulant kanye ne-antiphospholipid kungenziwa lapho:

• Ukuqubuka kwegazi okungalindelekile kwenzeka, njengabantu abasha noma labo abangenazo ezinye izinto eziyingozi ekunqandeni kwegazi.
• Owesifazane unomlando wokulahlekelwa kokukhulelwa okuphindaphindiwe.

Ukuhlolwa kwe-lupus anticoagulant ukuhlolwa kwegazi. Ama-antibodies e-antiphospholipid (aPL) abangela ukuthi ukuhlolwa kube okungajwayelekile elebhu.

Izinhlobo zokuhlolwa kwe-clotting zingafaka:

• Isikhathi esisebenzayo se-thromboplastin esinqunyiwe (aPTT)
• Isikhathi sobuthi sikaRussell viper
• Ukuhlolwa kokuvinjelwa kwe-Thromboplastin

Kuzokwenziwa futhi izivivinyo zama-antiphospholipid antibodies (aPL). Kubandakanya:

• Ukuhlolwa kwe-Anticardiolipin antibody
• Ama-antibody ku-beta-2-glypoprotein I (Beta2-GPI)

Umhlinzeki wakho wezokunakekelwa kwempilo uzoxilonga i-antiphospholipid antibody syndrome (APS) uma une-positive test ye-APL noma i-lupus anticoagulant, kanye nomcimbi owodwa noma eminingi elandelayo:

• Ukuqina kwegazi
• Izisu eziphindaphindwayo

Ukuhlolwa okuqondile kudinga ukuqinisekiswa ngemuva kwamasonto ayi-12. Uma unokuhlolwa okuhle ngaphandle kwezinye izici zesifo, ngeke uthole ukuxilongwa kwe-APS.

Ukwelashwa kwe-APS kuhloselwe ukuvimbela izinkinga ezivela kumahlule amasha egazi akha noma amahlule akhona akhule ngokwengeziwe. Uzodinga ukuthatha uhlobo oluthile lomuthi wokunciphisa igazi. Uma futhi unesifo sokuzilimaza, njenge-lupus, kuzodingeka ugcine leso simo silawulwa futhi.

Ukwelashwa okuqondile kuzoya ngokuthi isimo sakho sinzima kangakanani kanye nobunzima obudalayo.

I-ANTIPHOSPHOLIPID ANTIBODY SYNDROME (APS)

Ngokuvamile, uzodinga ukwelashwa nge-thinner yegazi isikhathi eside uma une-APS. Ukwelashwa kokuqala kungaba yi-heparin. Le mithi inikezwa ngomjovo.

Ezimweni eziningi, i-warfarin (i-Coumadin), enikezwa ngomlomo, iyaqalwa. Kuyadingeka ukuqapha njalo izinga le-anticoagulation. Lokhu kuvame ukwenziwa kusetshenziswa ukuhlolwa kwe-INR.

Uma une-APS futhi ukhulelwa, kuzodingeka ulandelwe eduze ngumhlinzeki oyingcweti kulesi simo. Ngeke uthathe i-warfarin ngesikhathi sokukhulelwa, kepha uzonikezwa isibhamu se-heparin esikhundleni salokho.

Uma une-SLE ne-APS, umhlinzeki wakho uzoncoma nokuthi uthathe i-hydroxychloroquine.

Njengamanje, ezinye izinhlobo zemithi yokuncipha kwegazi azinconywa.

ICATASTROPHIC ANTIPHOSPHOLIPID SYNDROME (CAPS)

Ukwelashwa kwe-CAPS okubandakanya inhlanganisela yokwelashwa kwe-anticoagulation, imithamo ephezulu yama-corticosteroids, kanye nokushintshaniswa kwe-plasma kube ngempumelelo kubantu abaningi. Kwesinye isikhathi i-IVIG, rituximab noma i-eculizumab nayo isetshenziselwa amacala abucayi.

ISIVIVINYO ESIHLE SE-LUPUS ANTICOAGULANT NOMA APL

Ngeke udinge ukwelashwa uma ungenazo izimpawu, ukulahlekelwa ukukhulelwa, noma uma ungakaze ube negazi.

Thatha izinyathelo ezilandelayo ukusiza ukuvimbela amahlule egazi ekwakhiweni:

• Gwema amaphilisi amaningi okulawula ukubeletha noma izindlela zokwelapha ama-hormone zokunqamuka kokuya esikhathini (abesifazane).
• UNGABHALI noma usebenzise eminye imikhiqizo kagwayi.
• Sukuma uzule phakathi nezindiza ezindiza ezinde noma ngezinye izikhathi lapho kufanele uhlale noma ulale isikhathi eside.
• Hambisa amaqakala akho phezulu naphansi lapho ungakwazi ukunyakaza.

Uzonikezwa imithi yokuncipha igazi (efana ne-heparin ne-warfarin) ukusiza ukuvimbela amahlule egazi:

• Ngemuva kokuhlinzwa
• Ngemuva kokuphuka kwethambo
• Ngomdlavuza osebenzayo
• Lapho udinga ukuhlala noma ukulala phansi isikhathi eside, njengesikhathi sokuhlala esibhedlela noma ukululamela ekhaya

Kungase kudingeke futhi ukuthi uthathe izinciphisi zegazi amasonto amathathu kuye kwayi-4 ngemuva kokuhlinzwa ukuze wehlise ubungozi bamahlule egazi.

Ngaphandle kokwelashwa, abantu abane-APS bazophinda bavale futhi. Isikhathi esiningi, umphumela muhle ngokwelashwa okufanele, okubandakanya ukwelashwa kwesikhathi eside kwe-anticoagulation. Abanye abantu bangaba namahlule egazi okunzima ukuwalawula naphezu kokwelashwa. Lokhu kungaholela kwi-CAPS, engabeka impilo yakho engcupheni.

Shayela umhlinzeki wakho uma ubona izimpawu ze-clot yegazi, njenge:

• Ukuvuvukala noma ukubomvu emlenzeni
• Ukuphelelwa umoya
• Ubuhlungu, ukuba ndikindiki, nombala wesikhumba ophaphathekile engalweni noma emlenzeni

Futhi khuluma nomhlinzeki wakho uma ulahlekelwe yisisu kaningi (ukukhulelwa kwesisu).

Ama-antibodies e-Anticardiolipin; I-Hughes syndrome

• Ukuqubuka kwesistimu ye-lupus erythematosus ebusweni
• Amahlule egazi

U-Amigo MC, uKhamashta MA. I-Antiphospholipid syndrome: i-pathogenesis, ukuxilongwa, nokuphathwa. Ku: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, abahleli. I-Rheumatology. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2019: isahluko 148.

UCervera R, uRodríguez-Pintó I, uColafrancesco S, et al. I-14th International Congress nge-Antiphospholipid Antibodies Task Force ibika ngesifo esiyinhlekelele se-antiphospholipid syndrome. Yenza ngokuzenzakalela uMfu. 2014; 13 (7): 699-707. I-PMID: 24657970 www.ncbi.nlm.nih.gov/pubmed/24657970.

UDufrost V, Risse J, Wahl D, Zuily S. Direct anticoagulants oral use in antiphospholipid syndrome: ingabe lezi zidakamizwa ziyindlela ephumelelayo nephephile kune-warfarin? Ukubuyekezwa okuhlelekile kwezincwadi: impendulo kumazwana. ICurr Rheumatol Rep. 2017; 19 (8): 52. I-PMID: 28741234 www.ncbi.nlm.nih.gov/pubmed/28741234.

U-Erkan D, uSalmon JE, uLokshin MD. Isifo se-anti-phospholipid. Ku: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, abahleli. Incwadi kaKelley noFirestein yeRheumatology. Umhlaka 10. IPhiladelphia, PA: Elsevier; 2017: isahluko 82.

Iwebhusayithi yeNational Heart, Lung, neGazi Institute. I-Antiphospholipid antibody syndrome. www.nhlbi.nih.gov/health-topics/antiphospholipid-antibody-syndrome. Kufinyelelwe ngoJuni 5, 2019.