Ðe nèdi hã be yeanya nu tso dɔléle sia si mebɔ o ŋua? Mina míaƒo nu tso EGPA (Eosinophilic Granulomatosis With Polyangiitis) ŋu le nya bɔbɔewo me!

Ðe asrã le ŋuwò enuenua? Alo ɖe nènye amesi ŋu nulɔdɔ sesẽwo le? Ekema nya si gblɔ ge magblɔ ate ŋu anɔ vevie na wò ŋutɔ. Elabena egbea míele nu ƒom tso dɔléle aɖe si wɔ nuku vie eye mebɔ kura o si do ƒome kple nɔnɔme mawo ɣeaɖewoɣi ŋu. Woyɔa dɔléle sia be EGPA . Woyɔe tsã be Churg-Strauss ƒe dɔléle . Ne edze abe ŋkɔa didi vie ene la, mègatsi dzi o, mina míase egɔme bɔbɔe.

Nukae nye EGPA (Eosinophilic Granulomatosis Kple Polyangiitis)?

Ŋkɔ didi sia ate ŋu atɔtɔ ame vie, gake mina míamae ɖe akpa vovovowo me eye míase egɔme. Ekema enɔa bɔbɔe wu sã.

1. Eosinophilic ƒe dɔléle

Kpuie ko la, eosinophilic fia be ʋumenugbagbevi ɣi ƒomevi aɖe si woyɔna be eosinophils ƒe xexlẽme sɔ gbɔ wu alesi wòle le wò ʋu me. Bu eŋu ale, eosinophils le abe dzɔla suewo ene le míaƒe ŋutilã me, wokpena ɖe míaƒe dɔlélenutsiŋutete ŋu . Gake le amesi ŋu EGPA le gome la, eosinophils ƒe xexlẽme ate ŋu adzi ɖe edzi zi gbɔ zi eve, alo agbɔ xexlẽme si sɔ gɔ̃ hã ŋu ɣeaɖewoɣi. Esia fia be dɔlélenutsiŋutete le dɔ wɔm gaƒoƒo geɖe wu.

2. Granulomatosis ƒe dɔléle

Granulomatosis nye nɔnɔme si me dɔlélenutsiŋutete ƒe lãmenugbagbeviwo wɔa ƒu suesuesuewo le ŋutilã ƒe lãkusiwo me ne dɔdzẽ, alo vuvu le eŋu. Míeyɔa ƒunukpeƒetetedɔ siawo be granulomas . Esia hã nye dɔlélenutsiŋutete ƒe dɔ. Ele abe ɖe nèƒo xlã teƒe si kuxi aɖe le eye nèle agbagba dzem be yeaxe mɔ nɛ be wòagakaka o ene.

3. Polyangiitis ƒe dɔléle

Polyangiitis nye ʋuka geɖe siwo le wò ŋutilã me ƒe dɔléle si nɔa anyi didi, vevietɔ esiwo le sue kple esiwo lolo (poly gɔmee nye geɖe). Enye nɔnɔme si le dɔlélewo ƒe hatsotso gã aɖe si woyɔna be vasculitis me . Angiitis nye ŋkɔ bubu na ʋukawo me dɔléle.

Eyata ne wotsɔ nusiawo katã ƒo ƒui la, EGPA nye nɔnɔme si mebɔ o si tsoa dɔlélenutsiŋutete ƒe dɔwɔwɔ si mesɔ o gbɔ si dzea egɔme tso asrã alo nulɔdɔwo dzi, hea eosinophils ƒe dzidziɖedzi vɛ, granulomas ƒe wɔwɔme, eye mlɔeba ʋukawo ƒe dɔdzẽ vɛ.

Aleke EGPA ƒe nɔnɔme sia kpɔa ŋusẽ ɖe nye ŋutilã dzii?

Ne ʋukawo ƒe dɔdzẽ yi edzi, si woyɔna be vasculitis , la, ʋukawo ƒe gliwo gbɔdzɔna. Esia ateŋu ana woakeke eye woado abe ʋuƒo ene – si míeyɔna be ʋuka me ʋu ƒe sisi . Alo, ʋuka siawo siwo gbɔdzɔ ate ŋu agbã eye ʋu nado ɖe ŋutilãa me.

Nu bubue nye be dɔdzẽ ate ŋu ana ʋukawo navuvu, abi nanɔ wo ŋu, eye woƒe ememe natsi. Nukae dzɔna ɣemaɣi? Ʋu meganɔa sisim o, eye ɣeaɖewoɣi gɔ̃ hã xea mɔ nɛ keŋkeŋ. Esia wɔnɛ be ɔksidzin kple nunyiame siwo ŋutilã hiã be wòate ŋu awɔ dɔ nyuie la meganɔa eŋu o.

Esi wònye be polyangiitis gblẽa nu le ʋuka sue siwo le míaƒe ŋutilã katã me ŋu ta la, dzesiawo ate ŋu adze le ŋutilãa ƒe akpa sia akpa. Gake zi geɖe la, egblẽa nu le gbɔgbɔtsixe , si fia be lãkusiwo ŋu. Ate ŋu agblẽ nu le ayiku, dɔgbo, dzi, kple lãmekawo hã ŋu.

Nukae nye EGPA ƒe dzesiwo?

Dɔléle sia si woyɔna be EGPA medzena zi ɖeka o. Dzesiawo dzena vivivi, le ƒe geɖe me, le afɔɖeɖe vovovowo me. Na míakpɔ nusiwo nye afɔɖeɖe mawo ɖa.

Afɔɖeɖe 1: Dzesi siwo bɔ siwo wokpɔna le eƒe gɔmedzedze

Le ɣeyiɣi sia me la, àte ŋu ase nusiwo le abe:

• Asrã si dzea egɔme tso ame tsitsiwo me: Ame si mexɔ asrã kpɔ o gɔ̃ hã ate ŋu axɔ asrã yeye si ƒe dzesiwo abe kɔdzi, ʋuʋudedi, kple gbɔgbɔ sesẽ ene.
• Hay fever: Aɖuɖɔɖɔɖɔ, ŋɔti me ʋuʋu, kple ŋɔti me ʋuʋu ate ŋu anɔ anyi.
• Sinusitis si nɔa anyi didi: Ta ƒe kpekpe kple nyaƒoɖeamenu ƒe seselelãme le esi sinus ƒe dowo ƒe dɔdzẽ ta.
• Nasal polyps: Nu suesuesue siwo menye kansa o siwo dzɔna le ŋɔti me.
• Ŋutilã ƒe vevesese le goawo katã me: Ne èse le ɖokuiwò me be yeƒe ƒunukpeƒewo le veve sem la, wò lãmekawo le veve sem.
• Asrã, lãmetutudɔ, kple ɖeɖiteameŋu: Ðeko vevesese kple ɖeɖiteameŋu.

Afɔɖeɖe Evelia: Eosinophils ƒe Dzidziɖedzi ƒe Dzesiwo

Ne dɔlélea le sesẽm ɖe edzi la, eosinophil mawo siwo ŋu míeƒo nu tsoe la dzea ƒoƒo ƒu ɖe ŋutilã ƒe lãkusi vovovowo me, vevietɔ le lãkusiwo me. Woyɔa esia be eosinophilic infiltrates . Esia ate ŋu ahe dzesi yeyewo vɛ:

• Akɔtave kple gbɔgbɔtsixe ƒe dzidziɖedzi.
• Dzi ƒe tsotso.
• Ŋutilã me ʋuʋudedi alo teƒeteƒewo.
• Nuɖuɖumeŋusẽ ƒe dzesiwo: nuwo abe dɔgbo ƒe vevesese, aɖuɖɔtoe ene.

Afɔɖeɖe Etɔ̃lia: Vasculitis ƒe Dzesiwo

Zi geɖe la, ʋukawo ƒe dɔléle , si nye ʋukawo ƒe dɔdzẽ, ye nyea dɔlélea ƒe akpa mamlɛtɔ. Esia ate ŋu ahe dzesi sesẽwo abe:

• Ʋu le dodom le ememe ƒe dzesiwo: Ʋu le aɖuɖɔ me, ʋu le kɔdzi, kple teƒe siwo ƒe amadede trɔ le ŋutigbalẽa te.
• Dzesi siwo tsoa dɔdzẽ alo lãmekawo ƒe gbegblẽ gbɔ: abɔ kple afɔwo ƒe ʋuʋu, ʋuʋudedi, gbɔdzɔgbɔdzɔ, lãmekawo ƒe vevesese.
• Dzidɔ ƒe dzesiwo: dzi ƒe tsotso si mesɔ o, alɔ̃madɔmadɔ, gbɔgbɔtsixe tsia ame ƒo, vuvu (edema) .

Esia nye polyangiitis.Nɔnɔmea ate ŋu agblẽ nu le ayikuwo hã ŋu, gake zi geɖe la, edzɔna dzesi aɖeke manɔmee.

Nukatae EGPA sia doa mo ɖa? Nuka tae?

Le nyateƒe me la, mía ŋutɔwo ƒe dɔlélenutsiŋutete ƒe dɔmawɔmawɔ nyuie gbɔe EGPA sia tsona . Bu eŋu kpɔ, nusi le dzɔdzɔm le afisia le abe asrafo siwo wobu be woakpɔ míaƒe ŋutilã ta, siwo le woawo ŋutɔ tɔ dzi dzem ene. Dɔdzẽ si le wò ʋukawo me, granulomas mawo siwo ŋu míeƒo nu tsoe va yi ƒe wɔwɔ, eosinophils ƒe dzidziɖedzi – esiawo katã dzɔna elabena dɔlélenutsiŋutete wɔa dɔ akpa eye wòwɔa nu masɔmasɔ.

Gake numekulawo mete ŋu le nusita tututu nusia dzɔna la kpɔm kokoko o.

Woyɔa dɔléle siwo me dɔlélenutsiŋutete ƒe dɔdzẽ ɣeyiɣi didi le ɣeaɖewoɣi be dɔlélenutsiŋutete ƒe dɔlélewo . Nusi bɔ ɖe dɔléle mawo ŋue nye be wowɔa dɔlékuiwutike siwo tsia tre ɖe lãmenugbagbevi aɖewo ŋu le ŋutilãa me hedzea wo dzi. Esia ate ŋu anye nusiwo gbɔ EGPA tsona dometɔ ɖeka.

Numekulawo ƒo dɔléle bubu geɖe abe EGPA ene nu ƒu ɖe hatsotso ɖeka me. Dɔlékuiwutike aɖe koŋ le wo katã si si woyɔna be ANCA . Woyɔa ƒuƒoƒo sia be ANCA-associated vasculitis (AAV) . Ke hã, amesiwo ŋu EGPA le ƒe 40% koe ANCA dɔlékuiwutike sia le. Esia fia be menye eya ɖeka kolia gbɔe wòtso o.

Edze abe eosinophils ƒe dzidziɖedzi nye nu bubu si hea dzesi siwo doa ŋgɔ na ʋukawo me dɔléle vɛ ene. Ne eosinophils ƒo ƒu ɖe lãkusiwo me la, wohea dɔdzẽ vɛ eye wògblẽa nu le lãkusiawo ŋu. Eosinophils ƒe dzidziɖedzi sia do ƒome vevie kple asrã kple nulɔdɔwo. Amesiwo ŋu asrã le ate ŋu axɔ EGPA zi gbɔ zi 34 wu ame bubuwo.

Nukawoe nye kuxi sesẽ siwo ate ŋu ado mo ɖa le EGPA ta?

Ne womeda dɔléle sia nyuie o la, ate ŋu agblẽ nu le wò ŋutinuwo kple lãmekawo ŋu vevie. Kpɔɖeŋu ʋɛ aɖewoe nye esi:

• Pleural effusion: Lãmetsi siwo ƒoa ƒu ɖe lãkusiwo ŋu.
• Pericardial effusion: Lãmetsi siwo ƒoa ƒu ɖe dzia ŋu.
• Ahɔhɔ̃mekawo ƒe dɔléle: Nusiwo gblẽa nu le lãmeka siwo le gota gome ŋu.
• Ayikudɔ si nɔa anyi didi.
• Yagbɔgbɔ ƒe dɔmawɔmawɔ nyuie atraɖii.
• Dzi ƒe dɔmawɔmawɔ nyuie.

Nu vevitɔe nye be woakpɔ dɔlélea ahadae hafi kuxi siawo tɔgbe nado mo ɖa.

Aleke wowɔa dea dzesi EGPA?

EGPA didi ate ŋu anye ayemɔ vie, elabena dzesi vovovowo ate ŋu adze le ŋuwò le ɣeyiɣi vovovowo me. Ate ŋu axɔ ɣeyiɣi aɖe hafi mia kple wò ɖɔkta miate ŋu aƒo wo katã nu ƒu. Azɔ hã, EGPA ƒe dzesiwo ate ŋu asɔ kple dɔléle bubu geɖewo ƒe dzesiwo.

Gake ɖɔkta ate ŋu asusu EGPA ne dɔléle siawo dometɔ geɖe le ŋuwò:

• Asrã alo hay fever ƒe ŋutinya.
• Ʋudodokpɔ ɖee fia be eosinophil ƒe xexlẽme dzi ɖe edzi.
• Nɔnɔmetatawo dodokpɔwo ɖe kpeɖodzi fia be eosinophilic infiltrates.
• Lãmetsiŋusẽ ŋuti numekuku ɖea kpeɖodzi fiana be granulomas kple/alo ʋukawo me dɔléle le eŋu.

Nukae nye EGPA dɔléle sia dada? Naneke meli si ŋu woavɔ̃ ɖo o, alo?

Ẽ, dɔléle sia ƒe atike aɖe li si woyɔna be EGPA godoo. Mega tsi dzi o! Nu vevitɔe nye be woakpɔ dɔlélea kaba eye woadze edada gɔme.

Zi geɖe la, nu gbãtɔ si woawɔe nye be woana corticosteroids agbɔsɔsɔ gã aɖe be wòaɖe dɔdzẽa kple eosinophils dzi akpɔtɔ . Ne atike siawo ɖu dzesiawo dzi la, míegblɔna be dɔlélea le ɖeɖem kpɔtɔ . Ema fia be dzesiawo nu yi. Emegbe ɖɔktaa ɖea atikeawo ƒe agbɔsɔsɔ dzi kpɔtɔna vivivi. Ele be ame geɖe nayi edzi anɔ corticosteroids nom le agbɔsɔsɔ sue aɖe me be woalé dɔléle sia ƒe tsɔtsɔke me ɖe asi.

Ɣeaɖewoɣi la, ɖewohĩ corticosteroid ɖeɖe dzaa masɔ gbɔ o, alo ahiã be nàyi edzi anɔ corticosteroids ƒe agbɔsɔsɔ gbogbo aɖe nom be nàƒo asa na eƒe nugbegblẽ le ame ŋu. Le nɔnɔme mawo me la, wò ɖɔkta ate ŋu aŋlɔ atike bubuwo na wò.

Atike bubu siawo dometɔ aɖewoe nye dɔlélenutsiŋutete ƒe atikewo kple dɔlékuiwutikewo . Mepolizumab nye dɔlékuiwutike gbãtɔ si dzi United States ƒe Nuɖuɖu Kple Atike Ŋuti Dɔwɔƒe (FDA) da asi ɖo na EGPA. Biologic , si nye benralizumab, hã ɖe nu nyuiwo fia le dɔdamɔnu dodokpɔ siwo wowɔ nyitsɔ laa me. Numekuku yeye sia ʋu atikewɔmɔnu yeyewo ɖi na amesiwo ŋu EGPA le.

Aleke agbea anɔ le EGPA gome? (Nyagblɔɖi) .

EGPA nye dɔléle si woate ŋu ada, gake womate ŋu adae o . Ame akpa gãtɔ ate ŋu atsɔ atikewo aɖu woƒe dzesiwo dzi eye woayi woƒe dɔlélea ƒe tsɔtsɔke me. Esia fia be dzesiawo nu yina. Gake ne èdzudzɔ atikewɔwɔ la, dzesiawo ate ŋu atrɔ ava (gbugbɔgadzɔ) . Eyata wò ɖɔkta alé ŋku ɖe wò nɔnɔmea ŋu le wò agbemeŋkekewo katã me.

Ðe wòanya wɔ be woanɔ agbe didi kple EGPAa?

Ne èda dɔ nyuie la, àte ŋu anɔ agbe si sɔ kple EGPA. Le dɔlélea ƒe akpa siwo de ŋgɔ me la, kuxiwo abe ŋutinuwo ƒe dɔmawɔmawɔ nyuie ene ate ŋu akpɔ ŋusẽ ɖe wò agbenɔƒe dzi. Gake zi geɖe la, atikewɔwɔ ate ŋu atɔ te ŋutinuwo ƒe dɔmawɔmawɔ nyuie alo atrɔe. Le atikewɔwɔ me la, ƒe atɔ̃ ƒe agbetsitsi ƒe agbɔsɔsɔme na EGPA wu 80%.

Ðe mɔ aɖe li si dzi woato axe mɔ na EGPA be wòagadze oa?

Esi míenya nusita tututu EGPA dzɔna haɖe o ta la, míenya alesi míaxe mɔ ɖe enu o. Gake ne wokpɔ dɔlélea kaba hedae ate ŋu akpe ɖe ame ŋu be nɔnɔmea nagagblẽ ɖe edzi o . Ne èlé ŋku ɖe wò dzesiwo ŋu nyuie eye nègblɔ wo na wò ɖɔkta la, àte ŋu akpe ɖe ŋuwò nàxe mɔ ɖe EGPA ƒe kuxi sesẽwo nu.

Aleke wòle be malé be na ɖokuinye esime mele agbe kple EGPA?

Esia nye nyabiase vevi aɖe ŋutɔ. Atike siwo wonana na EGPA, vevietɔ corticosteroids kple dɔlélenutsiŋutete , ɖea wò dɔlélenutsiŋutete dzi kpɔtɔna . Esia fia be anɔ bɔbɔe be nàdze dɔ, eye ne èdze dɔ la, exɔa ɣeyiɣi didi hafi wòhaya.

• Eyata ele be nàɖɔ ŋu ɖo wu alesi nèwɔnɛ ɖaa be nàƒo asa na dɔléle siwo bɔ . Kpɔ nyuie vevietɔ ne èle yiyim ɖe teƒe siwo amewo sɔ gbɔ ɖo kple teƒe siwo dɔnɔwo le.
• Klɔ asi nyuie enuenu.
• Ƒo nuɖuɖu si naa lãmesẽ.
• Zã ɣeyiɣi agbɔsɔsɔme si nèhiã be nàdɔ alɔ̃.
• Ne wozã corticosteroids ɣeyiɣi didi la, nugbegblẽ le ame ŋu abe sukli ƒe agbɔsɔsɔ fũ le ʋu me (abe suklidɔ ene), lolo, ƒuwo ƒe ƒuƒu (abe ƒumeŋuɖui ene), kple seselelãme ƒe tɔtrɔ ate ŋu ado mo ɖa . Ðo dze kple wò ɖɔkta tso nusiawo ŋu eye nàsrɔ̃ alesi nàkpɔ wo gbɔe. Gblɔe na wò ɖɔkta enumake ne dzesi yeye aɖewo dze le ŋuwò.

Mlɔeba la, nusiwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Eosinophilic granulomatosis with polyangiitis (EGPA) nye ʋukawo me dɔléle ƒomevi aɖe, eye ƒã ŋutɔ hã eye wòsesẽna be woakpɔe. Ðewohĩ dzesi vovovo siwo adze abe ɖe womedo ƒome kple wo nɔewo o ene la anɔ ŋuwò. Ate ŋu axɔ ɣeyiɣi aɖe hafi wò ɖɔkta nakpɔ nusi wofia ahatso nya me le atikewɔwɔ si nèhiã ŋu.

Nya nyuiae nye be woate ŋu akpɔ EGPA gbɔ nyuie ne wozãe . Ke hã, ahiã be nàwɔ tɔtrɔ aɖewo le wò gbesiagbegbenɔnɔ me. Agbenɔnɔ kple dɔléle si nɔa anyi didi si ŋu ame geɖe mese kpɔ o ate ŋu ase le eɖokui me ɣeaɖewoɣi be yetsi akogo. Le ɣeyiɣi siawo tɔgbe me la, ate ŋu aɖe vi be nàdo ka kple ame bubu siwo le EGPA ƒe kpekpeɖeŋunahabɔbɔwo. Mègana mɔkpɔkpɔ gbeɖe o, wɔ ɖe atikewɔlawo ƒe aɖaŋuɖoɖo nyuitɔ dzi, eye wò hã àte ŋu anɔ agbe nyui.

` EGPA, Churg-Strauss ƒe dɔléle, eosinophilic granulomatosis polyangiitis, asrã, nulɔdɔ, ʋukawo ƒe dɔléle, eosinophil