Ukukhubazeka ngokuzenzekelayo kuyabangela ukuba i-blood vessel ishawulwe
I-Granulomatosis kunye ne-polyangiitis (GPA), eyaziwa ngokuqhelekileyo njenge-granulomatosis ye-Wegener, yintlupheko engavumelekanga yokuzimela komzimba eyenza ukuvuvukala kwemithambo yegazi kwiindawo ezahlukeneyo zomzimba.
Izizathu
Njengokuba kukho konke ukuphazamiseka kweemoto, iGPA ibonakaliswe ngumzimba wokuzivikela omzimba. Ngenxa yezizathu ezingaziwa, umzimba uya kuphawula ngokuchanekileyo izicubu eziqhelekileyo kwimithambo yegazi njengangaphandle.
Ukuze udibanise isongelo esicatshulwayo, amaseli omzimba azakujikeleza iiseli aze enze i-nodule eqinile eyaziwayo njengegranuloma .
Ukubunjwa kwama-granulomas kunokukhokelela ekuphuhliseni ukuguquka okungapheliyo kwimithambo yegazi echaphazelekayo (imeko ebizwa ngokuba yi- vasculitis ). Ngokuhamba kwexesha, oku kunokwenza kube lula ukukhupha izitya kwaye kubangele ukuba baqhekeke, ngokuqhelekileyo kwisayithi ye-growth granulomatous. Inokubangela ukuba imithwalo yegazi ibe nzima kwaye incitshiswe, ukusika igazi kwiindawo ezibalulekileyo zomzimba.
I-GPA ithintela kakhulu imithwalo yegazi. Nangona iiphene zokuphefumula, imiphunga, kunye neentso zijoliswe kwiinjongo zokuhlaselwa, i-GPA ingabangela umonakalo kwesikhumba, amajoyina kunye nenkqubo yesifo. Intliziyo, ubuchopho, kunye nesisu somzimba asithintekiyo.
I-GPA ithinta amadoda kunye nabasetyhini ngokulinganayo, ngokukodwa phakathi kweminyaka engama-40 no-60. Kuthatyathwa njengesifo esingaqhelekanga kunye nesiganeko sonyaka kuphela malunga nama-10 ukuya kwe-20 kwiziganeko kwizigidi zabantu.
Iimpawu zokuqala kunye neempawu
Iimpawu ze-GPA ziyahluka ngendawo yokuvuvuka kwezicathulo. Kwizifo ezisekuqaleni, iimpawu zihlala zingacacanga kwaye zingezizo ezicacileyo ezifana neempumlo ze-runny, iintlungu zomzimba, ukunyunyuza, kunye ne-post-nasal drip.
Nangona kunjalo, njengoko eso sifo siqhubeka, ezinye, iimpawu ezibi kakhulu ziyakhula, kuquka:
- Ukuhla ukusinda
- Ukukhathala
- Ukuphelelwa kwesidlo
- Fever
- Iphunga legazi
- Iintlungu zesifuba (okanye ngaphandle kokuphefumula)
- Intlungu yentloko
Ubume obuqhelekileyo bale miqondiso kaninzi lukwazi ukuxilongwa. Akuqhelekanga, umzekelo, ukuba i-GPA ingachongwa kwaye iphathwe njengesifo sokuphefumula. Kuphela xa oogqirha bengenakufumana ubungqina bentsholongwane okanye i-bacterial kubangelwa uphando olungakumbi, ngakumbi xa kukho ubungqina be-vasculitis.
Iimpawu zeMpawu
Njengesifo sesistim, i-GPA inokubangela ukulimala omnye okanye iinkqubo ezininzi zegungu kanye. Nangona indawo yeempawu zikwazi ukuhluka, i-causes (vasculitis) ikwazi ukukhomba ugqirha ngokubhekiselele kwisifo sokuxilongwa ngokuzenzekelayo ukuba izitho ezininzi zibandakanyeka.
Iimpawu eziqhelekileyo zeGPA zingabandakanya:
- Ukuwa kwebhuloho yangasese ngenxa ye-septum ephazamisayo (eyaziwa nangokuthi "iphunga lekhanda" ukuguqulwa okufana nokubonwa kwexesha elide lokusebenzisa i-cocaine)
- Ukulahlekelwa ngamazinyo ngenxa yokutshatyalaliswa kwethambo
- Ukulahlekelwa kwindlebe yokuvalelwa kubangelwa ngumonakalo kwindlebe yangaphakathi
- Ukuphuhliswa kokukhula kwe-granulomatous kwiindawo zeso
- Izwi litshintsha ngenxa yokunciphisa umzila
- Igazi kwi-urine ( hematuria )
- Ukulahleka ngokukhawuleza kwentsholongwane ekhokelela ekuphelelweni kwezintso
- Isikhwehlo esinokugawula igazi ngenxa yokubunjwa kwezilonda ezinobunzima kunye nemivumba emiphakeni
- I-Arthritis (ngokuqhelekileyo ifunyanwe i- rheumatoid arthritis )
- Ukuphuhliswa kweebomvu ezibomvu okanye ezimfusa elukhumbeni ( purpura )
- Ubungqina, ukubetha, okanye ukutshisa okubangelwa yingozi yomonakalo (i- neuropathy )
Izindlela zokuxilonga
Ukuxilongwa kwe-GPA ngokuqhelekileyo kwenziwa kuphela emva kweempawu ezininzi ezingahambelaniyo zingachazi ixesha elide. Nangona kukho iimvavanyo zegazi ezifumanekayo ukufumanisa i- autoantibodies ezithile ezihambelana nezifo, ubukho (okanye ukungabikho) kwee-antibodies akwanele ukuqinisekisa (okanye ukugatya) ukuxilongwa.
Kunoko, ukuxilongwa kusetyenziswa ngokusetyenzwa kweempawu, iimvavanyo zebhabhi, ii-X ray, kunye neziphumo zovavanyo lomzimba.
Ezinye izixhobo zingadinga ukuxhasa ukuxilongwa, kubandakanywa ne-biopsy yeesiski ezichaphazelekayo. I- biopsy yepopu ngokuqhelekileyo yindawo efanelekileyo yokuqala nangona kungekho mpawu yokuphefumula. I-Biopsies ye-upper tractatory tract, ngokwahlukileyo, ithambekele ekubeni yincinci kunokuba i-50 ekhulwini ayiyi kubonisa iimpawu ze-granulomas okanye umonakalo weethambo.
Ngokufanayo, i-X-ray okanye i-CT scan ikhombisa ukubonisa ukungaqhelekanga kwemiphunga kubantu abanomsebenzi wamaphaphu.
Ngokubambisana, ukudibanisa iimvavanyo kunye neempawu zanele ngokwaneleyo ukuxhasa ukuxilongwa kwe-GPA.
Unyango lwangoku
Ngaphambi kwee-1970, i-granulomatosis ye-Wegener yayicatshulwa phantse yonke into ebulalayo, ngokuqhelekileyo ngenxa yokungaphumeleli kokuphefumula okanye uremia (imeko ebandakanya amanqanaba aphezulu kakhulu kwimveliso yenkunkuma kwigazi).
Kwiminyaka yakutshanje, ukudibanisa i- corticosteroid ye-high-dose kunye nezidakamizwa zokuxhatshazwa ngumzimba zibonakalise ukuphumelela kwi-75 ekhulwini yamatyala.
Ngokwenza ngokunyanisekileyo ukunciphisa ukuvuvukala kunye ne-corticosteroids kunye nokuchukumisa impendulo yokuzimela ngokuzikhusela nge-cyclophosphamide, abantu abaninzi abane-GPA bangaphila ubomi obude, benempilo kwaye bahlale bexolelwa iminyaka engama-20 okanye ngaphezulu.
Emva kokuqala unyango, i-corticosteroid dosages iyancitshiswa njengoko eso sifo silawulwa. Kwezinye iimeko, iziyobisi zinokumiswa ngokupheleleyo.
I-Cyclophosphamide, ngokuphambene, idla ngokumiselwa iinyanga ezintathu ukuya ezintandathu kwaye yatshintshela kwenye, i-immunosuppressant encinci. Ubungakanani bonyango lokulondolozwa luyahluka kodwa luhlala luphela unyaka okanye ezimbini ngaphambi kokuba kukho na utshintsho olwenziwe ngamanani.
Kubantu abanesifo esibi, ezinye, kungenelela ngoncedo olungakumbi, kuquka:
- I-dose ephakamileyo yonyango
- Ukutshintshiselwa kwePlasma (apho igazi lahlukana khona ukususa ii-autoantibodies)
- Ukufakelwa kweengso
Prognosis
Nangona kukho izinga eliphezulu lokuxolelwa, ukuya kuma-50 ekhulwini abantu abaphathwayo baya kuhlaselwa kwakhona. Ngaphezu koko, abantu abaneGPA basengozini yokuxhatshazwa kwexesha elide, kubandakanya ukungaphumeleli kwintsholongwane engapheliyo, ukulahlekelwa kwindlebe kunye nokuva. Indlela efanelekileyo yokukhusela oku kukuqwalasela rhoqo ugqirha wakho kunye nokuhlolwa kwegazi kunye nokuhlolwa kweengcinga.
Ngolawulo olufanelekileyo lwesifo, iipesenti ezingama-80 zezigulane eziphathwe ngempumelelo ziya kuphila iminyaka engama-8. I- anti-antier-based-treatment based and a penicillin-derivative ebizwa ngokuthi i- CellCept (i-mycophenolate mofetil) ingaqhubela phambili ukuphucula loo miphumo kwiminyaka ezayo.
> Imithombo:
> Almouhawis, H .; Leao, J; Fedele, S. kunye nePorter, S. "I-granulomatosis ye-Wegener: ukuhlaziywa kweempawu zekliniki kunye nokuhlaziywa kwi-diagnostic and treatment." Umbhalo weNgcaciso yomlomo. 2013; 42: 507-516.
> Fortin, P .; Tejani, A .; Bassett, K .; kunye no-Musini, V. "I-immunoglobulin engaphathekiyo ngaphezu kokunyanga okuqhelekileyo kwe-granulomatosis ye-Wegener." Cochrane Data Syst Rev. 2013; 1: I-DOI: 10.1002 / 14651858.CD007057.pub3
> Silva, S .; Iinkcukacha, U. Kaira, S. et al. "" I-Mycophenolate uMotiltil for Induction and Maintenance of Remission kwi-Microscopic Polyangiitis enobudlelwane obuncinane bokubandakanywa kwamaRenal-I-Pilot Trial Triel Trial. " Iklinikhi J Am Soc Nephrol. 2010; 5 (3): 445-453.