Ukukhubazeka okuzenzakalelayo kubangela ukugoba kwegazi kwesibindi
I-granulomatosis ne-polyangiitis (i-GPA), eyaziwa ngokuvamile njenge-granulomatosis ka-Wegener, iyisifo esingavamile sokuzimela komzimba esibangela ukuvuvukala kwemithambo yegazi ezingxenyeni ezihlukene zomzimba.
Izimbangela
Njengazo zonke izifo ezizimele, i-GPA ibonakala isistimu yomzimba ayikho. Ngezizathu ezingaziwa, umzimba uzodinga ngokungaqondile izicubu ezivamile emithanjeni yegazi njengangaphandle.
Ukuze uqukathe usongo olubonakalayo, amaseli omzimba azungeze amangqamuzana futhi ahlanganise nodule oluqinile olubizwa ngokuthi i- granuloma .
Ukwakhiwa kwe-granulomas kungaholela ekuthuthukiseni ukuvuvukala okungapheli emithanjeni yegazi ethintekayo (isimo esibizwa ngokuthi i- vasculitis ). Ngokuhamba kwesikhathi, lokhu kungahle kubenciphise izitsha futhi kubangele ukuba kuqhume, ngokuvamile endaweni yesimo se-granulomatous. Kungabangela futhi ukuthi imithwalo yegazi ibe nzima futhi ifinyelele, ukusika igazi egazini ezingxenyeni ezibalulekile zomzimba.
I-GPA ihlasela kakhulu imithwalo yegazi emincane. Ngenkathi isifo sokuphefumula, amaphaphu, nezinso kuyizinhlobonhlobo eziyinhloko zokuhlaselwa, i-GPA ingabangela nokulimala esikhunjeni, amalunga, nesistimu yezinzwa. Inhliziyo, ubuchopho, kanye namasongo omzimba akuvamile ukuthinteka.
I-GPA ithinta amadoda nabesifazane ngokulinganayo, ngokuyinhloko phakathi kweminyaka engama-40 no-60. Kubhekwa njengesifo esingavamile kanye nesigameko sonyaka samaphesenti angu-10 kuya kwangu-20 kuphela kubantu abayizigidi ezilodwa.
Izimpawu Zokuqala Nezibonakaliso
Izimpawu ze-GPA ziyahlukahluka ngendawo yokuvuvukala kwezicathulo. Esikhathini sesifo sokuqala, lezi zimpawu zivame ukungaqondakali futhi ezingacacile ezifana nekhala eligijimayo, ubuhlungu bomzimba, ukusiphaza, nokuvuza okumanzi.
Kodwa-ke, njengoba lesi sifo siqhubeka, ezinye, izimpawu ezingathí sina ezingase zikhule, kufaka phakathi:
- Ukulahlekelwa kwesisindo
- Ukukhathala
- Ukulahlekelwa kwesifiso
- Fever
- Iphunga liphuma
- Izinhlungu zesifuba (noma ngaphandle kokuphefumula)
- Ubuhlungu bendlebe ephakathi
Imvelo evamile yalezi zimpawu ngokuvamile ingenza ukuxilongwa kunzima. Kuyinto engavamile, isibonelo, ukuthi i-GPA ingaphathwa kabi futhi iphathwe njengesifo sokuphefumula. Kuphela lapho odokotela bengatholakali ubufakazi bokuthi i-viral noma ibhaktheriya kubangelwa ukuthi uphenyo oluqhubekayo lungalaywa, ikakhulukazi uma kukhona ubufakazi be-vasculitis.
Izimpawu zesimiso
Njengesifo sesistimu, i-GPA ingabangela ukulimala esisodwa noma izinhlelo eziningana zomzimba ngesikhathi esisodwa. Ngenkathi indawo yezimpawu zingashintsha, imbangela eyinhloko (i-vasculitis) ingavame ukukhomba udokotela ngokubhekisisa ukuhlolwa okuzenzakalelayo uma izitho eziningi zihilelekile.
Izimpawu zesistimu ze-GPA zingabandakanya:
- Ukuwa kwesibhuloho esiswini ngenxa ye-septum ephazamisekile (eyaziwa nangokuthi "iphunga lesikhumba" lesifana nokufana nokuboniswa kokusetshenziswa kwe-cocaine eside)
- Ukulahlekelwa izinyo ngenxa yokubhujiswa kwamathambo
- Ukulahleka kokuzwa okuzwakalayo kubangelwa ukulimala endlebeni yangaphakathi
- Ukuthuthukiswa kokukhula okukhululekile ezingxenyeni zeso
- Izinguquko zezwi ngenxa yokwehliswa kwe-trachea
- Igazi emcimbini (i- hematuria )
- Ukulahlekelwa okusheshayo kwezinso zezinso eziholela ekuhlulekeni kwezinso
- Ukukhwehlela nge-phlegm enegazi ngenxa yokwakheka kwezilonda ezincane nezigxobo emaphashini
- I-arthritis (evame ukutholwa ukuthi i- rheumatoid arthritis )
- Ukuthuthukiswa kwezimpawu ezibomvu noma ezibomvu esikhumbeni ( purpura )
- Ubungqingili, ukugubha, noma ukuvutha okubangelwa ukulimala kwamagciwane ( neuropathy )
Izindlela Zokuxilongwa
Ukuxilongwa kwe-GPA kuvame ukwenza kuphela emva kwezinyathelo eziningana, ezingavumelani okungaqondakali isikhathi eside. Ngenkathi kunezivivinyo zegazi ezitholakalayo ukuze zihloniphe ama-autoantibodies athile ahlotshaniswa nezifo, ukutholakala (noma ukungabi khona) kwamagciwane okuwanele okwanele ukuqinisekisa (noma ukwenqaba) ukuxilongwa.
Esikhundleni salokho, ukuxilongwa kwenziwa ngokusekelwe kokuhlanganiswa kwezimpawu, ukuhlolwa kwebhu, ama-X ray kanye nemiphumela yokuhlolwa ngokomzimba.
Amanye amathuluzi angadinga ukusekela ukuxilongwa, kufaka phakathi i-biopsy yezicubu ezithintekile. I- biopsy yamaphaphu ngokuvamile iyindawo engcono kakhulu yokuqala ngisho noma kungekho izimpawu zokuphefumula. I-Biopsies ye-upper tractatory tract, ngokuphambene, ijwayele ukusiza kakhulu kusukela amaphesenti angu-50 ngeke akhombise izimpawu ze-granulomas noma umonakalo wesisindo.
Ngokufanayo, i-X-ray yesifuba noma i-CT scan ingavame ukuveza ukungalungi kwamaphaphu kubantu abanezinye izinto ezivamile zamaphaphu.
Ngokubambisana, ukuhlanganiswa kwezivivinyo kanye nezimpawu kungase kwanele ukusekela ukuxilongwa kwe-GPA.
Ukwelashwa Kwamanje
Ngaphambi kweminyaka yama-1970, i-wegener's granulomatosis ibhekwa njengabantu abaningi ababulalayo, ngokuvamile ngenxa yokuhluleka ukuphefumula noma i- uremia (isimo esibandakanya amazinga aphezulu kakhulu emikhiqizo edoti egazini).
Eminyakeni yamuva, ukuhlanganiswa kwemithi ephezulu ye- corticosteroid kanye nezidakamizwa zokuzivikela ngegciwane lesandulela ngculaza kuye kwafakazela ukuthi kuphumelele ukukhishwa kwamacala ngamaphesenti angu-75 wamacala.
Ngokuzikhandla ekunciphiseni ukuvuvukala nge-corticosteroids nokuhlenga impendulo yokuziphendulela ngokuzibulala ngezidakamizwa ezithintekayo zokuzivikela njenge-cyclophosphamide, abantu abaningi abane-GPA bangaphila impilo ende, enempilo futhi bahlale bekhululekile iminyaka engu-20 noma ngaphezulu.
Ngemuva kokwelashwa kokuqala, imithi ye-corticosteroid ivame ukunciphisa njengoba lesi sifo silawulwa. Kwezinye izimo, izidakamizwa zingase zimiswe ngokuphelele.
I-Cyclophosphamide, ngokuphambene, ijwayele ukufakwa ezinyangeni ezintathu kuya kweziyisithupha bese ishintshela kwesinye isikhathi, esincane se-immunosuppressant. Isikhathi sokwelashwa kwesondlo singashintsha kepha ngokuvamile sihlala unyaka noma amabili ngaphambi kokuthi kuguqulwe noma yikuphi ukuguqulwa kwesilinganiso.
Kubantu abanesifo esinzima, okunye, ukungenelela okunokwedlulele kungadingeka, kuhlanganise:
- Ukwelapha okuphezulu okukhipha imithi
- Ukushintshaniswa kwe-plasma (lapho igazi lihlukaniswa ukuze lisuse ama-autoantibodies)
- Ukufakelwa kwezinso
Isibikezelo
Naphezu kwamazinga aphezulu okuxolelwa, amaphesenti angaba ngu-50 abantu abalashwa bazobhekana nokubuyela emuva. Ngaphezu kwalokho, abantu abane-GPA basengozini yezinkinga zesikhathi eside, kuhlanganise nokuhluleka kwezinso ezingapheli, ukulahlekelwa kwezindlebe nokuzwa. Indlela engcono kakhulu yokugwema lokhu ukuhlela ukuhlolwa njalo nodokotela wakho kanye nokuhlolwa okuvamile kwegazi nokucabanga.
Ngokuphathwa kahle kwalesi sifo, amaphesenti angu-80 weziguli eziphathwa ngempumelelo azophila iminyaka engaba ngu-8. Izindlela zokwelapha ezintsha zokulwa namagciwane kanye ne-penicillin-derivative ebizwa ngokuthi i- CellCept (mycophenolate mofetil) ingathuthukisa leyo miphumela eminyakeni ezayo.
> Imithombo:
> Almouhawis, H .; Leao, J .; Fedele, S. and Porter, S. "I-granulomatosis ka-Wegener: ukubuyekezwa kwezici zomtholampilo nokubuyekezwa kokuxilongwa nokuphathwa." I-Journal Oral Path Imithi. 2013; 42: 507-516.
> Fortin, P .; I-Tejani, A .; I-Bassett, K .; kanye no-Musini, V. "I-immunoglobulin engathandeki ngaphezu kwemithi ejwayelekile ye-granulomatosis ka-Wegener." I-Cochrane Data Syst Rev. 2013; I-1: DOI: 10.1002 / 14651858.CD007057.pub3
> Silva, S .; Izinkomba, U .; Kaira, S. et al. "" I-Mycophenolate i-Mofetil ye-Induction ne-Maintenance of Remission ku-microscopic Polyangiitis ngokubambisana okulula okulinganayo-okuyi-Apt-Label-Open Pilot Trial. " I-Clin J Am Soc Nephrol. 2010; 5 (3): 445-453.