Ngaba nawe uyafuna ukwazi ngesi sifo singaqhelekanga? Masithethe nge-EGPA (Eosinophilic Granulomatosis With Polyangiitis) ngokulula!

Ngaba unesifo sesifuba esihlala sikhona? Okanye ngaba ungumntu onesifo se-allergies esinzima? Oko ndiza kukuthetha kunokuba yinto ebalulekileyo kuwe. Kuba namhlanje siza kuthetha ngesifo esingaqhelekanga nesingaqhelekanga esidla ngokunxulunyaniswa neemeko ezinjalo. Esi sifo sibizwa ngokuba yi -EGPA . Sasisaziwa njenge -Churg-Strauss syndrome . Ukuba igama libonakala lide kancinci, ungakhathazeki, masiliqonde ngokulula.

Yintoni i-EGPA (i-Eosinophilic Granulomatosis enePolyangiitis)?

Eli gama lide linokuba lidida kancinci, kodwa masiliqhekeze libe ziinxalenye size siliqonde. Emva koko kulula kakhulu.

1. I-Eosinophilic

Ngamafutshane, i-eosinophilic ithetha ukuba unenani eliphezulu kuneliqhelekileyo lohlobo lweseli emhlophe yegazi ebizwa ngokuba yi-eosinophils egazini lakho. Cinga ngale ndlela, ii-eosinophils zifana nabagcini abancinci emizimbeni yethu, zinceda inkqubo yethu yokuzikhusela komzimba . Kodwa kumntu one-EGPA, inani lee-eosinophils ngamanye amaxesha linokuphinda kabini, okanye lidlule, inani eliqhelekileyo. Oku kuthetha ukuba inkqubo yokuzikhusela yomzimba isebenza ixesha elide.

2. I-Granulomatosis

I-Granulomatosis yimeko apho iiseli zomzimba zenza amaqhuma amancinci kwizicubu zomzimba xa kukho ukudumba, okanye ukudumba. La maqhuma siwabiza ngokuba yi-granulomas . Lo ngumsebenzi wenkqubo yomzimba yokuzikhusela. Kufana nokujikeleza indawo enengxaki nokuzama ukuyithintela ukuba ingasasazeki.

3. I-Polyangiitis

I-Polyangiitis kukudumba okuhlala ixesha elide kwemithambo yegazi emininzi emzimbeni wakho, ingakumbi emincinci nephakathi (i-poly ithetha ezininzi). Yimeko ekwiqela elikhulu lezifo ezibizwa ngokuba yi-vasculitis . I-Angiitis lelinye igama le-vasculitis.

Ngoko ke, konke oku xa kudityaniswa, i-EGPA yimeko engaqhelekanga ebangelwa kukungasebenzi kakuhle kwenkqubo yomzimba yokuzikhusela eqala nge-asthma okanye i-allergies, ekhokelela ekwandeni kwee-eosinophils, ukwakheka kwee-granulomas, kwaye ekugqibeleni ukudumba kwemithambo yegazi.

Le meko ye-EGPA iwuchaphazela njani umzimba wam?

Xa kukho ukudumba okuqhubekayo kwemithambo yegazi, okubizwa ngokuba yi-vasculitis , iindonga zemithambo yegazi ziba buthathaka. Oku kunokubangela ukuba zoluleke kwaye zidumbe njengebhaluni - esizibiza ngokuba yi-aneurysm . Okanye, le mithambo yegazi ibuthathaka inokuqhuma ize yophe emzimbeni.

Enye into kukuba ukudumba kunokubangela ukuba imithambo yegazi idumbe, ibe namanxeba, ize inciphe ngaphakathi. Kwenzeka ntoni ke ngoko? Ukuhamba kwegazi kuyaphazamiseka, ngamanye amaxesha kuthintelwe ngokupheleleyo. Oku kuthintela umzimba ukuba ungafumani ioksijini kunye nezondlo ezifunekayo ukuze usebenze kakuhle.

Ngenxa yokuba i-polyangiitis ichaphazela imithambo yegazi emincinci kuyo yonke imizimba yethu, iimpawu zinokwenzeka naphi na emzimbeni. Nangona kunjalo, idla ngokuchaphazela inkqubo yokuphefumla , oko kukuthi imiphunga. Ingachaphazela nezintso, amathumbu, intliziyo, kunye nemithambo-luvo.

Zithini iimpawu ze-EGPA?

Esi sifo esibizwa ngokuba yi-EGPA asibonakali ngequbuliso. Iimpawu zibonakala kancinci kancinci, kwiminyaka emininzi, ngamanqanaba ahlukeneyo. Masibone ukuba zeziphi ezo zigaba.

Inqanaba loku-1: Iimpawu eziqhelekileyo ezibonakala kwizigaba zokuqala

Ngeli xesha ungaziva izinto ezinje:

• Isifuba somntu omdala: Kwanomntu ongazange abe nesifuba somntu ngaphambili unokufumana isifo somntu esitsha sesifuba esineempawu ezifana nokukhohlela, ukuphefumla nzima, kunye nobunzima bokuphefumla.
• Umkhuhlane wengca: Ukuthimla, impumlo erhawuzelelwayo, kunye nokuxinana kwempumlo kusenokuqhubeka.
• I-Chronic Sinusitis: Imvakalelo yobunzima kunye noxinzelelo entloko ngenxa yokudumba kwemingxuma ye-sinus.
• Iipolyps zempumlo: Ziintlobo ezincinci, ezingengomhlaza ezikhula ngaphakathi empumlweni.
• Intlungu yomzimba ngokubanzi: Ukuziva ngathi amalungu akho ayabuhlungu, izihlunu zakho ziyaqaqamba.
• Umkhuhlane, ukudinwa, kunye nokudinwa: Ndiziva ndibuhlungu kwaye ndidiniwe.

Inqanaba lesiBini: Iimpawu zokwanda kwee-Eosinophils

Njengoko isifo siqhubeka, ezo eosinophils sithethe ngazo ziqala ukuqokelelana kwizicubu ezahlukeneyo zomzimba, ingakumbi imiphunga. Oku kubizwa ngokuba yi-eosinophilic infiltrates . Oku kunokukhokelela kwiimpawu ezintsha:

• Intlungu esifubeni kunye nobunzima bokuphefumla obandayo.
• Ukubetha kwentliziyo.
• Amaqhuqhuva okanye amabala esikhumba.
• Iimpawu zenkqubo yokugaya ukutya: izinto ezinje ngentlungu yesisu, urhudo.

Inqanaba lesithathu: Iimpawu zeVasculitis

I-Vasculitis , oku kukudumba kwemithambo yegazi, idla ngokuba linqanaba lokugqibela lesi sifo. Oku kunokubangela iimpawu ezinzima ezifana nezi:

• Iimpawu zokopha ngaphakathi: Igazi elisesitulweni, igazi ekukhohleleni, kunye namabala amnyama phantsi kolusu.
• Iimpawu ezibangelwa kukudumba okanye umonakalo kwimithambo-luvo: ukungaziva malungu, ukurhawuzelelwa, ubuthathaka, iintlungu zemithambo-luvo.
• Iimpawu zesifo sentliziyo: ukubetha kwentliziyo okungahambelaniyo, ukuquleka, ukuphefumla okufutshane, ukudumba (i-edema) .

Le yi -polyangiitis.Le meko inokuchaphazela nezintso, kodwa uninzi lwexesha iyenzeka ngaphandle kweempawu.

Kutheni le nto kuvela le EGPA? Siyintoni isizathu?

Enyanisweni, le EGPA ibangelwa kukungasebenzi kakuhle kwenkqubo yethu yokuzikhusela komzimba . Khawuthelekelele, okwenzekayo apha kufana namajoni afanele ukukhusela imizimba yethu, ehlasela eyawo. Ukuvuvukala kwimithambo yegazi yakho, ukwakheka kwezo granulomas sithethe ngazo ngaphambili, ukwanda kwee-eosinophils - konke oku kwenzeka ngenxa yokuba inkqubo yokuzikhusela yomzimba isebenza kakhulu kwaye ayiphenduli ngendlela engafanelekanga.

Nangona kunjalo, abaphandi abakakwazi ukuqonda ukuba kutheni oku kusenzeka.

Izifo ezibandakanya ukudumba kwexesha elide kwenkqubo yomzimba yokuzikhusela ngamanye amaxesha zibizwa ngokuba zizifo zokuzikhusela komzimba . Uphawu oluqhelekileyo lwezifo ezinjalo kukuba ii-antibodies ziveliswa kwaye zihlasela iiseli ezithile emzimbeni. Oku kusenokuba yenye yezizathu ze-EGPA.

Abaphandi badibanise ezinye izifo ezininzi ezifana ne-EGPA kwiqela elinye. Zonke zine-antibody ethile ebizwa ngokuba yi-ANCA . Eli qela libizwa ngokuba yi-ANCA-associated vasculitis (AAV) . Nangona kunjalo, yi-40% kuphela yabantu abane-EGPA abanale antibody ye-ANCA. Oku kuthetha ukuba ayisiyo yodwa imbangela.

Ukwanda kwee-eosinophils kubonakala ngathi yimbangela eyahlukileyo yeempawu ezandulela i-vasculitis. Xa ii-eosinophils ziqokelelana kwizicubu, zibangela ukudumba kwaye zonakalise izicubu. Oku kwanda kwee-eosinophils kunxulunyaniswa kakhulu ne-asthma kunye ne-allergies. Abantu abane-asthma banamathuba angama-34 okuba ne-EGPA kunabanye.

Ziziphi iingxaki ezinkulu ezinokubakho ngenxa ye-EGPA?

Ukuba esi sifo asinyangwa kakuhle, sinokubangela umonakalo omkhulu kumalungu omzimba nakwizicwili zakho. Nazi imizekelo embalwa:

• Ukukhupha ulwelo olujikeleze imiphunga: Ukuqokelelana kolwelo olujikeleze imiphunga.
• Ukuphuma kwegazi entliziyweni: Ukuqokelelana kolwelo olujikeleze intliziyo.
• I-peripheral neuropathy: Ukonakala kwemithambo-luvo engaphandle.
• Isifo sezintso esingapheliyo.
• Ukungasebenzi kakuhle kokuphefumla okungapheliyo.
• Ukungaphumeleli kwentliziyo okubangelwa kukunganyamezelani.

Eyona nto ibalulekileyo kukufumanisa nokunyanga esi sifo ngaphambi kokuba kubekho iingxaki ezinje.

Ifunyaniswa njani i-EGPA?

Ukuchonga i-EGPA kunokuba nzima kancinci, kuba unokuba neempawu ezahlukeneyo ngamaxesha ahlukeneyo. Kungathatha ixesha ukuba wena nogqirha wakho nizidibanise zonke ezi zinto. Kwakhona, iimpawu ze-EGPA zinokufana neempawu zezinye izifo ezininzi.

Nangona kunjalo, ugqirha unokurhanela i-EGPA ukuba unezinye zezi meko:

• Imbali yesifo sesifuba okanye umkhuhlane wengca.
• Uvavanyo lwegazi lubonisa ukuba inani le-eosinophil liphezulu.
• Uvavanyo lwemifanekiso lubonisa ubungqina bokungena kwe-eosinophilic.
• I-biopsy yezicubu ibonisa ubungqina be-granulomas kunye/okanye i-vasculitis.

Luluphi unyango lwesi sifo se-EGPA? Akukho nto yokoyika, akunjalo?

Ewe, ngokuqinisekileyo lukho unyango lwale meko olubizwa ngokuba yi-EGPA. Ungakhathazeki! Eyona nto iphambili kukufumanisa isifo ngokukhawuleza uze uqale unyango.

Ngokwesiqhelo, into yokuqala ekufuneka yenziwe kukunika iidosi eziphezulu ze -corticosteroids ukunciphisa ukudumba kunye nee -eosinophils . Xa la mayeza elawula iimpawu, sithi isifo siyaphela . Oko kuthetha ukuba iimpawu ziphelile. Emva koko ugqirha uyayinciphisa kancinci idosi yamayeza. Abantu abaninzi kufuneka baqhubeke bethatha ii-corticosteroids ngedosi ephantsi ukuze bagcine olu xolo.

Ngamanye amaxesha ii-corticosteroids zodwa zisenokunganeli, okanye unokufuna ukuqhubeka uthatha iidosi eziphezulu ze-corticosteroids ukuze uphephe iziphumo ebezingalindelekanga. Kwimeko ezinjalo, ugqirha wakho unokukunika amanye amayeza.

La mayeza ongezelelweyo aquka amayeza okunciphisa amasosha omzimba kunye ne -biologics . I-Mepolizumab yeyona nto yokuqala evunyiweyo yi-US Food and Drug Administration (FDA) ye-EGPA. Enye i-biologic , i-benralizumab, ikwabonise iziphumo ezithembisayo kuvavanyo lweklinikhi lwakutshanje. Olu phando lutsha luvule iindlela ezintsha zonyango kubantu abane-EGPA.

Buza kuba njani ubomi nge-EGPA? (Ingxelo yokuqikelela)

I-EGPA sisifo esinokunyangwa, kodwa asinyangeki . Uninzi lwabantu lunokulawula iimpawu zabo ngamayeza baze babuyele esiqhelweni. Oku kuthetha ukuba iimpawu ziyaphela. Nangona kunjalo, ukuba uyeka unyango, iimpawu zinokubuya (ziphinde zibuye) . Ngoko ugqirha wakho uya kujonga imeko yakho ubomi bakho bonke.

Ngaba kunokwenzeka ukuba uphile ixesha elide ne-EGPA?

Ngonyango olusebenzayo, ungaphila ubomi obuqhelekileyo nge-EGPA. Kwinqanaba eliphezulu lesi sifo, iingxaki ezifana nokungasebenzi kakuhle kwamalungu omzimba zinokuchaphazela ubomi bakho. Nangona kunjalo, unyango lunokuyeka okanye lubuyisele ukungasebenzi kakuhle kwamalungu omzimba. Ngonyango, izinga lokusinda kweminyaka emihlanu kwi-EGPA lingaphezulu kwe-80%.

Ngaba ikho indlela yokuthintela i-EGPA ekuphuhliseni?

Ekubeni singazi kakuhle ukuba kutheni i-EGPA ikhula, asazi ukuba singayithintela njani. Nangona kunjalo, ukuxilongwa kwangoko kunye nonyango kunokunceda ukuthintela imeko ukuba ingabi mandundu . Ngokunika ingqalelo esondeleyo kwiimpawu zakho kwaye uzixele kugqirha wakho, unokunceda ukuthintela iingxaki ezinkulu ze-EGPA.

Ndingazinyamekela njani xa ndihlala ne-EGPA?

Lo ngumbuzo obaluleke kakhulu. Amayeza anikwa i-EGPA, ngakumbi ii-corticosteroids kunye ne -immunosuppressants , anciphisa amasosha omzimba wakho . Oku kuthetha ukuba unokuba sengozini yokugula, kwaye ukuba uyagula, kuthatha ixesha elide ukuba uphile.

• Ngoko ke, kufuneka ulumke ngakumbi kunesiqhelo ukuze uphephe izifo eziqhelekileyo . Lumka ngakumbi xa usiya kwiindawo ezixineneyo nakwiindawo apho kukho abantu abagulayo.
• Hlamba izandla zakho kakuhle rhoqo.
• Yitya ukutya okunempilo.
• Zinike ixesha elaneleyo lokulala.
• Xa ii-corticosteroids zithathwa ixesha elide, iziphumo ebezingalindelekanga ezifana namanqanaba aphezulu eswekile egazini (njengeswekile), ukunyuka kobunzima, ukuncitshiswa kwamathambo (njenge-osteoporosis), kunye notshintsho lweemvakalelo zinokwenzeka . Thetha nogqirha wakho ngezi zinto kwaye ufunde indlela yokuzilawula. Xelela ugqirha wakho ngoko nangoko ukuba uneempawu ezintsha.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Eosinophilic granulomatosis ene-polyangiitis (EGPA) luhlobo lwe-vasculitis, kwaye ikwanqabile kakhulu kwaye kunzima ukuyixilonga. Usenokuba neempawu ezahlukeneyo ezibonakala zinganxulumananga. Kungathatha ixesha ukuba ugqirha wakho afumanise ukuba zithetha ukuthini aze agqibe ukuba loluphi unyango olufunekayo.

Iindaba ezimnandi zezokuba i-EGPA inokulawulwa kakuhle ngonyango . Nangona kunjalo, kuya kufuneka wenze utshintsho oluthile kubomi bakho bemihla ngemihla. Ukuphila nesifo esinganyangekiyo abantu abaninzi abangazange beve ngaso ngamanye amaxesha kunokuziva unesizungu. Ngamaxesha anje, kunokuba luncedo ukunxibelelana neenethiwekhi zenkxaso zabanye abantu abaphila ne-EGPA. Ungaze ulahle ithemba, landela iingcebiso zonyango ezifanelekileyo, kwaye nawe ungaphila ubomi obuhle.

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