Uyafuna ukwazi ngalesi sifo esingavamile? Ake sixoxe nge-EGPA (Eosinophilic Granulomatosis With Polyangiitis) ngamazwi alula!

Ingabe unesifo sofuba esivame ukuba khona? Noma ungumuntu ohlushwa yi-allergies enzima? Khona-ke engizokusho kungaba yinto ebaluleke kakhulu kuwe. Ngoba namuhla sizokhuluma ngesifo esingajwayelekile nesingavamile kakhulu esivame ukuhlotshaniswa nezimo ezinjalo. Lesi sifo sibizwa ngokuthi i-EGPA . Ngaphambilini sasaziwa ngokuthi i-Churg-Strauss syndrome . Uma igama libonakala lide kancane, ungakhathazeki, masiliqonde kalula.

Kuyini i-EGPA (i-Eosinophilic Granulomatosis ene-Polyangiitis)?

Leli gama elide lingase lidideke kancane, kodwa ake silihlukanise libe izingxenye futhi siliqonde. Khona-ke kulula kakhulu.

1. I-Eosinophilic

Kalula nje, i-eosinophilic isho ukuthi unenani eliphakeme kunelijwayelekile lohlobo lweseli elimhlophe legazi elibizwa ngokuthi ama-eosinophils egazini lakho. Cabanga ngale ndlela, ama-eosinophils afana nabaqaphi abancane emizimbeni yethu, asiza isimiso sethu somzimba sokuzivikela . Kodwa kumuntu one-EGPA, inani lama-eosinophils ngezinye izikhathi lingaphindeka kabili, noma lidlule, inani elijwayelekile. Lokhu kusho ukuthi isimiso somzimba sokuzivikela sisebenza isikhathi esengeziwe.

2. I-granulomatosis

I-Granulomatosis yisimo lapho amangqamuzana omzimba akha khona amaqhubu amancane ezicutshini zomzimba uma kukhona ukuvuvukala, noma ukuvuvukala. Sibiza la maqhubu ngokuthi ama-granuloma . Lokhu futhi ngumsebenzi wesimiso somzimba sokuzivikela. Kufana nokuzungeza indawo enenkinga nokuzama ukuyivimba ukuthi ingasabalali.

3. I-Polyangiitis

I-Polyangiitis ukuvuvukala kwesikhathi eside kwemithambo yegazi eminingi emzimbeni wakho, ikakhulukazi emincane nephakathi nendawo (i-poly isho eminingi). Kuyisimo esiyingxenye yeqembu elikhulu lezifo ezibizwa ngokuthi i-vasculitis . I-Angiitis elinye igama le-vasculitis.

Ngakho-ke, konke lokhu uma kuhlanganisiwe, i-EGPA iyisimo esingavamile esibangelwa ukusebenza okungajwayelekile kwesimiso somzimba sokuzivikela esiqala nge-asthma noma i-allergies, okuholela ekwandeni kwama-eosinophils, ukwakheka kwama-granuloma, futhi ekugcineni ukuvuvukala kwemithambo yegazi.

Lesi simo se-EGPA sithinta kanjani umzimba wami?

Uma kukhona ukuvuvukala okuqhubekayo kwemithambo yegazi, okubizwa ngokuthi i-vasculitis , izindonga zemithambo yegazi ziba buthakathaka. Lokhu kungabangela ukuthi zeluleke futhi zikhukhumale njengebhaluni – esikubiza ngokuthi i-aneurysm . Noma, le mithambo yegazi ebuthakathaka ingaqhuma futhi yophe emzimbeni.

Enye into ukuthi ukuvuvukala kungabangela ukuba imithambo yegazi ivuvuke, ibe nezibazi, futhi ibe mncane ngaphakathi. Kwenzekani-ke? Ukugeleza kwegazi kuyaphazamiseka, ngezinye izikhathi kuze kuvalwe ngokuphelele. Lokhu kuphuca umzimba umoya-mpilo kanye nezakhamzimba ozidingayo ukuze usebenze kahle.

Ngenxa yokuthi i-polyangiitis ithinta imithambo yegazi emincane kuyo yonke imizimba yethu, izimpawu zingavela noma kuphi emzimbeni. Kodwa-ke, ivame ukuthinta uhlelo lokuphefumula , okusho amaphaphu. Ingathinta nezinso, amathumbu, inhliziyo, kanye nemizwa.

Ziyini izimpawu ze-EGPA?

Lesi sifo esibizwa ngokuthi i-EGPA asiveli ngokuzumayo. Izimpawu zivela kancane kancane, eminyakeni eminingi, ngezigaba eziningana. Ake sibone ukuthi ziyini lezo zigaba.

Isigaba 1: Izimpawu ezivamile ezibonakala ezigabeni zokuqala

Phakathi nalesi sikhathi ungase uzizwe izinto ezifana nalezi:

• I-asthma eqala kubantu abadala: Ngisho nomuntu ongakaze abe ne-asthma ngaphambili angaba ne-asthma entsha enezimpawu ezifana nokukhwehlela, ukuphefumula kanzima, kanye nobunzima bokuphefumula.
• Umkhuhlane we-hay: Ukuthimula, ikhala elilumayo, kanye nokuminyana kwamakhala kungase kuqhubeke.
• I-Sinusitis Engapheli: Umuzwa wokusinda kanye nokucindezeleka ekhanda ngenxa yokuvuvukala kwemigodi yama-sinus.
• Ama-polyp ekhala: Amahlumela amancane, angewona umdlavuza akhula ngaphakathi kwekhala.
• Ubuhlungu bomzimba obujwayelekile: Uzizwa sengathi amalunga akho ayabuhlungu, imisipha yakho iyabuhlungu.
• Umkhuhlane, ubuthakathaka, kanye nokukhathala: Ukuzizwa nje udabukile futhi ukhathele.

Isigaba Sesibili: Izimpawu Zokwanda Kwama-Eosinophil

Njengoba lesi sifo siqhubeka, lawo ma-eosinophil esikhulume ngawo aqala ukuqongelela ezicutshini ezahlukahlukene zomzimba, ikakhulukazi emaphashini. Lokhu kubizwa ngokuthi ama-eosinophilic infiltrates . Lokhu kungaholela ezimpawini ezintsha:

• Ubuhlungu besifuba kanye nobunzima obandayo bokuphefumula.
• Ukushaya kwenhliziyo.
• Ukuqubuka kwesikhumba noma amabala.
• Izimpawu zesistimu yokugaya ukudla: izinto ezinjengobuhlungu besisu, isifo sohudo.

Isigaba Sesithathu: Izimpawu ze-Vasculitis

I-Vasculitis , okuwukuvuvukala kwemithambo yegazi, ngokuvamile kuyisigaba sokugcina salesi sifo. Lokhu kungabangela izimpawu ezinzima ezifana nalezi:

• Izimpawu zokopha ngaphakathi: Igazi esitulweni, igazi ekukhwehleleni, kanye namabala anombala ngaphansi kwesikhumba.
• Izimpawu ezibangelwa ukuvuvukala noma ukulimala kwemizwa: ukungazweli ezithweni, ukuqaqamba, ubuthakathaka, ubuhlungu bemizwa.
• Izimpawu zesifo senhliziyo: ukushaya kwenhliziyo okungajwayelekile, ukuquleka, ukuphelelwa umoya, ukuvuvukala (i-edema) .

Lokhu kuyi -polyangiitis.Lesi simo singathinta nezinso, kodwa isikhathi esiningi senzeka ngaphandle kwezimpawu.

Kungani kuvela le-EGPA? Iyini imbangela?

Eqinisweni, le-EGPA ibangelwa ukungasebenzi kahle kwesimiso sethu somzimba sokuzivikela . Cabanga nje, okwenzekayo lapha kufana namasosha okufanele avikele imizimba yethu, ehlasela eyawo. Ukuvuvukala emithanjeni yegazi yakho, ukwakheka kwalawo ma-granuloma esikhulume ngawo ngaphambili, ukwanda kwama-eosinophils - konke lokhu kwenzeka ngoba isimiso somzimba sokuzivikela sisebenza ngokweqile futhi asisabeli kahle.

Kodwa-ke, abacwaningi abakakwazi ukuthola ukuthi kungani lokhu kwenzeka.

Izifo ezihilela ukuvuvukala kwesikhathi eside kwesimiso somzimba sokuzivikela komzimba ngezinye izikhathi zibizwa ngokuthi izifo zokuzivikela komzimba . Isici esivamile salezi zifo ukuthi ama-antibodies akhiqizwa ngokumelene namaseli athile emzimbeni futhi ahlasele. Lokhu kungaba yimbangela eyodwa ye-EGPA.

Abacwaningi bahlanganise ezinye izifo eziningana njenge-EGPA zaba yiqembu elilodwa. Zonke zine-antibody ethile ebizwa ngokuthi i-ANCA . Leli qembu libizwa ngokuthi i-ANCA-associated vasculitis (AAV) . Kodwa-ke, abantu abangu-40% kuphela abane-EGPA abanale antibody ye-ANCA. Lokhu kusho ukuthi akuyona yodwa imbangela.

Ukwanda kwama-eosinophil kubonakala kuyimbangela ehlukile yezimpawu ezandulela i-vasculitis. Lapho ama-eosinophil eqoqana ezicutshini, abangela ukuvuvukala futhi alimaze izicubu. Lokhu kwanda kwama-eosinophil kuhlotshaniswa kakhulu ne-asthma kanye nokungezwani komzimba. Abantu abane-asthma banamathuba aphindwe ka-34 okuthuthukisa i-EGPA kunabanye.

Yiziphi izinkinga ezinkulu ezingase zivele ngenxa ye-EGPA?

Uma lesi sifo singelashwa kahle, singadala umonakalo omkhulu ezithweni nasezicutshini zakho. Nazi izibonelo ezimbalwa:

• Ukuphuma kwegazi: Ukuqongelela koketshezi emaphashini.
• Ukuphuma kwegazi enhliziyweni: Ukuqongelela koketshezi oluzungeze inhliziyo.
• I-peripheral neuropathy: Ukulimala kwemizwa yangaphakathi.
• Isifo sezinso esingamahlalakhona.
• Ukwehluleka kokuphefumula okungapheli.
• Ukwehluleka kwenhliziyo okubangelwa ukuminyana.

Into ebaluleke kakhulu ukuxilonga nokwelapha lesi sifo ngaphambi kokuba kuvele izinkinga ezinjengalezi.

I-EGPA ihlolwa kanjani?

Ukuxilonga i-EGPA kungaba nzima kancane, ngoba ungaba nezimpawu ezahlukene ngezikhathi ezahlukene. Kungathatha isikhathi ukuthi wena nodokotela wakho nikuhlanganise konke. Futhi, izimpawu ze-EGPA zingafana nezimpawu zezinye izifo eziningi.

Kodwa-ke, udokotela angase asole i-EGPA uma unezimo eziningana zalezi:

• Umlando we-asthma noma umkhuhlane we-hay.
• Ukuhlolwa kwegazi kubonisa inani eliphakeme le-eosinophil.
• Ukuhlolwa kwezithombe kubonisa ubufakazi bokungena kwe-eosinophilic.
• Ukuhlolwa kwezicubu kubonisa ubufakazi be-granulomas kanye/noma i-vasculitis.

Iyini indlela yokwelapha lesi sifo se-EGPA? Akukho okufanele ukwesabe, akunjalo?

Yebo, kukhona ukwelashwa kwalesi simo okubizwa nge-EGPA. Ungakhathazeki! Into eyinhloko ukuxilonga lesi sifo ngokushesha bese uqala ukwelashwa.

Ngokuvamile, into yokuqala okufanele uyenze ukunikeza imithamo ephezulu yama -corticosteroids ukunciphisa ukuvuvukala nama -eosinophils . Uma le mithi ilawula izimpawu, sithi lesi sifo sesiphelile . Lokho kusho ukuthi izimpawu seziphelile. Udokotela ube esenciphisa kancane kancane umthamo womuthi. Abantu abaningi badinga ukuqhubeka nokuthatha ama-corticosteroids ngomthamo ophansi ukuze balondoloze lokhu kuphumula.

Ngezinye izikhathi ama-corticosteroids kuphela angase anganele, noma kungadingeka uqhubeke nokuthatha imithamo ephezulu yama-corticosteroids ukuze ugweme imiphumela emibi. Ezimweni ezinjalo, udokotela wakho angase akunikeze imithi eyengeziwe.

Le mithi eyengeziwe ifaka phakathi ama-immunosuppressants kanye nama-biologics . I-Mepolizumab iwumuthi wokuqala we-biologic ovunyelwe yi-US Food and Drug Administration (FDA) we-EGPA. Omunye umuthi we-biologic , i-benralizumab, nawo ubonise imiphumela ethembisayo ezivivinyweni zakamuva zemitholampilo. Lolu cwaningo olusha luvule izinketho ezintsha zokwelapha kubantu abane-EGPA.

Impilo izoba njani nge-EGPA? (Isibikezelo)

I-EGPA iyisifo esingelapheka, kodwa esingelapheki . Iningi labantu lingakwazi ukulawula izimpawu zabo ngemithi bese liqala ukudamba. Lokhu kusho ukuthi izimpawu ziyaphela. Kodwa-ke, uma uyeka ukwelashwa, izimpawu zingabuya (ziphinde zibuye) . Ngakho udokotela wakho uzoqapha isimo sakho impilo yakho yonke.

Kungenzeka yini ukuphila isikhathi eside ne-EGPA?

Ngokwelashwa okuphumelelayo, ungaphila impilo evamile nge-EGPA. Ezigabeni ezithuthukile zesifo, izinkinga ezifana nokwehluleka kwezitho zingathinta impilo yakho yonke. Kodwa-ke, ukwelashwa kuvame ukumisa noma ukubuyisela emuva ukwehluleka kwezitho. Ngokwelashwa, izinga lokusinda kweminyaka emihlanu le-EGPA lingaphezu kuka-80%.

Ingabe ikhona indlela yokuvimbela i-EGPA ukuthi ingakhuli?

Njengoba singakazi kahle ukuthi kungani i-EGPA ikhula, asazi ukuthi singayivimbela kanjani. Kodwa-ke, ukuxilongwa kusenesikhathi kanye nokwelashwa kungasiza ekuvimbeleni isimo ukuba singabi sibi kakhulu . Ngokunaka kakhulu izimpawu zakho nokuzibika kudokotela wakho, ungasiza ekuvimbeleni izinkinga ezinkulu ze-EGPA.

Kufanele ngizinakekele kanjani ngenkathi ngiphila ne-EGPA?

Lona umbuzo obaluleke kakhulu. Imithi enikezwa i-EGPA, ikakhulukazi ama-corticosteroids kanye nama-immunosuppressants , yehlisa amasosha omzimba wakho . Lokhu kusho ukuthi usengozini enkulu yokugula, futhi uma ugula, kuthatha isikhathi eside ukululama.

• Ngakho-ke, kudingeka uqaphele kakhulu kunokujwayelekile ukuze ugweme izifo ezivamile . Qaphela ikakhulukazi uma uya ezindaweni ezinabantu abaningi nezindawo lapho kukhona khona abantu abagulayo.
• Geza izandla zakho kahle njalo.
• Yidla ukudla okunempilo.
• Zinike isikhathi esiningi ngangokunokwenzeka ukuze ulale.
• Uma kuthathwa ama-corticosteroids isikhathi eside, imiphumela emibi efana namazinga aphezulu kashukela egazini (njengesifo sikashukela), ukukhuluphala, ukuncipha kwamathambo (njenge-osteoporosis), kanye nokushintsha kwemizwelo kungenzeka . Khuluma nodokotela wakho ngalokhu futhi ufunde ukuthi ungazilawula kanjani. Tshela udokotela wakho ngokushesha uma unezimpawu ezintsha.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

I-granulomatosis ye-eosinophilic ene-polyangiitis (EGPA) uhlobo lwe-vasculitis, futhi ayivamile futhi kunzima ukuyixilonga. Ungase ube nezimpawu ezahlukahlukene ezibonakala zingahlobene. Kungathatha isikhathi ukuthi udokotela wakho athole ukuthi zisho ukuthini futhi anqume ukuthi yiluphi ukwelashwa okudingayo.

Izindaba ezinhle ukuthi i-EGPA ingaphathwa kahle ngokwelashwa . Kodwa-ke, kuzodingeka wenze izinguquko ezithile empilweni yakho yansuku zonke. Ukuphila nesifo esingamahlalakhona abantu abaningi abangakaze bezwe ngaso ngezinye izikhathi kungazizwa unesizungu. Ngezikhathi ezinjengalezi, kungaba usizo ukuxhumana namanethiwekhi okusekela abanye abaphila ne-EGPA. Ungalokothi ulahle ithemba, landela iseluleko sezokwelapha esifanele, nawe ungaphila impilo enhle.

I- EGPA, i-Churg-Strauss syndrome, i-eosinophilic granulomatosis polyangiitis, i-asthma, i-allergy, i-vasculitis, i-eosinophil