I-Granulomatosis kunye ne-polyangiitis

I-Granulomatosis ene-polyangiitis (i-GPA) sisifo esinqabileyo apho imithambo yegazi idumbileyo. Oku kukhokelela kumonakalo kumalungu aphambili omzimba. Yayisaziwa njenge-Wegener’s granulomatosis.

I-GPA ikakhulu ibangela ukudumba kwemithambo yegazi emiphungeni, kwizintso, empumlweni, ezonweni nasezindlebeni. Oku kubizwa ngokuba yi-vasculitis okanye i-angiitis. Ezinye iindawo zinokuchaphazeleka kwezinye iimeko. Esi sifo sinokubulala kwaye unyango olukhawulezileyo lubalulekile.

Kwiimeko ezininzi, esona sizathu asaziwa, kodwa sisifo esingumzimba. Rhoqo, i-vasculitis ene-antineutrophil cytoplasmic antibodies (ANCA) ibangelwe ngamachiza aliqela kubandakanya i-cocaine esikwe nge-levamisole, hydralazine, propylthiouracil, kunye ne-minocycline.

I-GPA ixhaphake kakhulu kubantu abadala abaneminyaka ephakathi kumantla eYurophu. Inqabile ebantwaneni.

I-sinusitis rhoqo kunye neempumlo ezinegazi zezona mpawu zixhaphakileyo. Ezinye iimpawu zakuqala zibandakanya umkhuhlane ongenasizathu sicacileyo, ukubila ebusuku, ukudinwa, kunye nokugula ngokubanzi (malaise).

Ezinye iimpawu eziqhelekileyo zinokubandakanya:

• Izifo ezingapheliyo
• Iintlungu, kunye nezilonda ezijikeleze ukuvulwa kwempumlo
• Khohlela okanye ngaphandle kwegazi kwisikhohlela
• Intlungu yesifuba kunye nokuphefumla okufutshane njengoko isifo siqhubeka
• Ukuphelelwa ngumdla wokutya kunye nokwehla kobunzima
• Ulusu lutshintsha njengemigruzuko kunye nezilonda kulusu
• Iingxaki zezintso
• Umchamo wegazi
• Iingxaki zamehlo ukusuka kwi-conjunctivitis emnene ukuya kukudumba okukhulu kweliso.

Iimpawu eziqhelekileyo ziquka:

• Intlungu edibeneyo
• Ubuthathaka
• Intlungu zesisu

Unokuba novavanyo lwegazi olujonga iiproteni ze-ANCA. Olu vavanyo lwenziwa kubantu abaninzi abaneGPA esebenzayo. Nangona kunjalo, olu vavanyo ngamanye amaxesha lubi, nakubantu abanemeko.

I-x-ray yesifuba iya kwenziwa ukukhangela iimpawu zesifo semiphunga.

Uhlalutyo lomchamo lwenziwa ukujonga iimpawu zesifo sezintso ezifana neprotein kunye negazi kumchamo. Ngamanye amaxesha umchamo uqokelelwa ngaphezulu kweeyure ezingama-24 ukukhangela ukuba izintso zisebenza njani.

Uvavanyo lwegazi oluqhelekileyo lubandakanya:

• Gcwalisa ubalo lwegazi (CBC)
• Iphaneli yemetabolism ebanzi
• Izinga le-sedimentation ye-Erythrocyte (ESR)

Uvavanyo lwegazi lunokwenziwa ukukhuphela ecaleni ezinye izifo. Oku kunokubandakanya:

• Iintsholongwane zenyukliya
• I-anti-glomerular basement membrane (anti-GBM) izilwa-buhlungu
• C3 kunye C4, cryoglobulins, hepatitis serologies, HIV
• Uvavanyo lomsebenzi wesibindi
• Isikrini sesifo sephepha kunye neenkcubeko zegazi

Ngamanye amaxesha i-biopsy iyadingeka ukuqinisekisa ukuxilongwa nokujonga ukuba isifo sinzima kangakanani. I-biopsy yezintso iqhele ukwenziwa. Unokuba nakho oku kulandelayo:

• Impumlo ye-mucosal biopsy
• Vula i-lung biopsy
• Ulusu lwe-biopsy
• I-biopsy ephezulu yomoya

Olunye uvavanyo olunokwenziwa lunokubandakanya:

• Isinus CT scan
• Isifuba se-CT scan

Ngenxa yobume obunokubaluleka kwe-GPA, unokulaliswa esibhedlele. Nje ukuba kufunyaniswe isifo, uya kunyangwa ngeedosi eziphezulu ze-glucocorticoids (ezinje nge-prednisone). Ezi zinikezelwa ngomthambo iintsuku ezi-3 ukuya kwezi-5 ekuqaleni konyango. IPrednisone inikwa kunye namanye amayeza anciphisa ukuphendula komzimba.

Kwisifo esibuhlungu amanye amayeza anciphisa ukuphendula komzimba njengemethotrexate okanye i-azathioprine inokusetyenziswa.

• IRituximab (Rituxan)
• ICyclophosphamide (iCytoxan)
• Imethotrexate
• Azathioprine (Imuran)
• IMycophenolate (Cellcept okanye iMyfortic)

La mayeza ayasebenza kwisifo esibi, kodwa kunokubangela iziphumo ebezingalindelekanga ezimandundu.Uninzi lwabantu abane-GPA baphathwa ngamayeza aqhubekayo ukuthintela ukubuyela umva okungenani iinyanga ezili-12 ukuya kwezi-24. Thetha nomboneleli wakho wezempilo malunga nesicwangciso sakho sonyango.

Amanye amayeza asetyenziswa kwi-GPA aquka:

• Amayeza okuthintela ukulahleka kwethambo okubangelwa yi-prednisone
• Folic acid okanye ifilic acid, ukuba uthatha i-methotrexate
• Iintsholongwane zokuthintela usulelo lwemiphunga

Amaqela enkxaso nabanye abaphethwe zizifo ezifanayo banokunceda abantu abanale meko kunye neentsapho zabo ukuba zifunde ngezifo kwaye zilungelelanise notshintsho olunxulumene nonyango.

Ngaphandle konyango, abantu abaneendlela ezinzima zesi sifo banokusweleka kwiinyanga ezimbalwa.

Ngonyango, umbono wezigulana ezininzi ulungile. Uninzi lwabantu abafumana i-corticosteroids kunye namanye amayeza acothisa ukuphendula komzimba abe ngcono. Uninzi lwabantu abane-GPA baphathwa ngamayeza aqhubekayo ukuthintela ukubuyela umva kangangeenyanga ezili-12 ukuya kwezi-24.

Iingxaki zihlala zenzeka xa isifo singanyangwa. Abantu abane-GPA bahlakulela umonakalo wezicubu kwimiphunga, i-airways kunye neentso. Ukubandakanyeka kwezintso kunokubangela igazi kumchamo kunye nokusilela kwezintso. Isifo sezintso sinokuba mandundu ngokukhawuleza. Umsebenzi wezintso usenokungaphucuki nokuba imeko ilawulwa ngamayeza.

Ukuba ayinyangwa, ukusilela kwezintso kwaye mhlawumbi nokufa kwenzeka kwiimeko ezininzi.

Ezinye iingxaki zinokubandakanya:

• Ukudumba kwamehlo
• Ukungaphumeleli kwemiphunga
• Ukukhohlela igazi
• I-Nasal septum perforation (umngxuma ngaphakathi kwempumlo)
• Iziphumo ebezingalindelekanga ezivela kumayeza asetyenziselwa ukunyanga esi sifo

Fowunela umnikezeli wakho ukuba:

• Ukhula iintlungu esifubeni kunye nokuphefumla okufutshane.
• Ukhohlela igazi.
• Unegazi kumchamo wakho.
• Unazo ezinye iimpawu zesi sifo.

Akukho sithintelo saziwayo.

Ngaphambili: i-Wegener's granulomatosis

• I-Granulomatosis ene-polyangiitis emlenzeni
• Inkqubo yokuphefumla

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IPagnoux C, uGuillevin L; Iqela lesiFrentshi leVasculitis Study; MAINRITSAN abaphandi. Rituximab okanye ulondolozo lwe-azathioprine kwi-vasculitis enxulumene ne-ANCA. N Engl J Med. Ngo-2015; 372 (4): 386-387. IINKCUKACHA: 25607433 pubmed.ncbi.nlm.nih.gov/25607433/.

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I-Yates M, i-Watts RA, i-Bajema IM, okqhubekayo. Iingcebiso ze-EULAR / ERA-EDTA kulawulo lwe-vasculitis enxulumene ne-ANCA. [ulungiso olupapashiweyo luyavela kwi U-Ann Rheum Dis. 2017;76(8):1480]. U-Ann Rheum Dis. Ngo-2016; 75 (9): 1583-1594. IINKCUKACHA: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.