I-Granulomatosis ene-polyangiitis

I-Granulomatosis ne-polyangiitis (GPA) yisifo esingajwayelekile lapho imishanguzo yegazi ivuvuka khona. Lokhu kuholela ekulimaleni ezithweni ezinkulu zomzimba. Beyaziwa phambilini njenge-Wegener’s granulomatosis.

I-GPA ikakhulu ibangela ukuvuvukala kwemithambo yegazi emaphashini, izinso, impumulo, amasinusi nezindlebe. Lokhu kubizwa nge-vasculitis noma i-angiitis. Ezinye izindawo zingathinteka kwezinye izimo. Lesi sifo singabulala futhi ukwelashwa ngokushesha kubalulekile.

Ezimweni eziningi, imbangela ngqo ayaziwa, kepha kuyisifo esizimele. Imvamisa, i-vasculitis ene-antineutrophil cytoplasmic antibodies (i-ANCA) ibangelwe yimithi eminingi ehlanganisa i-cocaine esikwe nge-levamisole, i-hydralazine, i-propylthiouracil, ne-minocycline.

I-GPA ivame kakhulu kubantu abadala abaneminyaka ephakathi nendawo abavela enyakatho yeYurophu. Kuyaqabukela ezinganeni.

I-sinusitis ejwayelekile namakhala anegazi yizimpawu ezivame kakhulu. Ezinye izimpawu zokuqala zifaka umkhuhlane ongenasizathu esicacile, ukujuluka ebusuku, ukukhathala, kanye nokugula okujwayelekile (malaise).

Ezinye izimpawu ezivamile zingafaka:

• Ukutheleleka okungapheli ezindlebeni
• Ubuhlungu, nezilonda ezungeze imbobo yekhala
• Khwehlela ngegazi noma ngaphandle kwesikhwehlela
• Ubuhlungu besifuba nokuphefumula okuncane njengoba lesi sifo siqhubeka
• Ukwehla kwesifiso sokudla nokuncipha
• Izinguquko zesikhumba ezinjengemihuzuko nezilonda zesikhumba
• Izinkinga zezinso
• Umchamo onegazi
• Izinkinga zamehlo kusuka ku-conjunctivitis emnene kuya ekuvuvukaleni okukhulu kweso.

Izimpawu ezingavamile kakhulu zifaka:

• Ubuhlungu obuhlangene
• Ubuthakathaka
• Ubuhlungu besisu

Ungaba nokuhlolwa kwegazi okubheka amaprotheni e-ANCA. Lezi zivivinyo zenziwa kubantu abaningi abane-GPA esebenzayo. Kodwa-ke, lokhu kuhlolwa kwesinye isikhathi akubi kahle, ngisho nakubantu abanalesi sifo.

Kuzokwenziwa i-x-ray yesifuba ukubheka izimpawu zesifo samaphaphu.

Ukuhlolwa komchamo kwenziwa ukubheka izimpawu zesifo sezinso ezifana namaprotheni negazi emchameni. Kwesinye isikhathi umchamo uqoqwa ngaphezu kwamahora angama-24 ukubheka ukuthi izinso zisebenza kanjani.

Ukuhlolwa kwegazi okujwayelekile kufaka:

• Qedela ukubalwa kwegazi (CBC)
• Iphaneli yemethodi ephelele
• Izinga le-sedimentation ye-Erythrocyte (ESR)

Ukuhlolwa kwegazi kungenziwa ukukhipha ezinye izifo. Lokhu kungafaka:

• Amasosha omzimba aphikisayo
• Ama-anti-glomerular basement membrane (anti-GBM) amasosha omzimba
• I-C3 ne-C4, ama-cryoglobulins, i-hepatitis serologies, i-HIV
• Ukuhlolwa kokusebenza kwesibindi
• Isikrini sofuba namasiko egazi

Ngezinye izikhathi kudingeka i-biopsy ukuqinisekisa ukuxilongwa nokuhlolwa ukuthi isifo sinzima kangakanani. I-biopsy yezinso yenziwa kakhulu. Ungase futhi ube nokukodwa kokulandelayo:

• I-Nasal mucosal biopsy
• Vula i-biopsy yamaphaphu
• Isikhumba biopsy
• I-biopsy ephezulu yomoya

Olunye uvivinyo olungenziwa lubandakanya:

• Isinema CT scan
• Isikena se-CT esifubeni

Ngenxa yesimo esibi kakhulu se-GPA, ungahle ulaliswe esibhedlela. Lapho ukuxilongwa sekwenziwe, mhlawumbe uzophathwa ngemithamo ephezulu ye-glucocorticoids (efana ne-prednisone). Lokhu kunikezwa ngomthambo izinsuku ezi-3 kuye kwezi-5 ekuqaleni kokwelashwa. IPrednisone inikezwa kanye neminye imithi eyehlisa ijubane ukuphendula komzimba.

Ngesifo esibi kakhulu eminye imithi eyehlisa ijubane ukuphendula kwamagciwane njenge-methotrexate noma i-azathioprine ingasetshenziswa.

• Isi-Rituximab (Rituxan)
• ICyclophosphamide (iCytoxan)
• IMethotrexate
• I-Azathioprine (Imuran)
• I-Mycophenolate (Cellcept noma iMyfortic)

Le mithi iyasebenza ezifweni ezinzima, kepha ingadala imiphumela emibi kakhulu.Iningi labantu abane-GPA baphathwa ngemithi eqhubekayo yokuvikela ukubuyela emuva okungenani izinyanga eziyi-12 kuye kwangama-24. Khuluma nomhlinzeki wakho wezokunakekelwa kwempilo ngohlelo lwakho lokwelashwa.

Eminye imithi esetshenziselwa i-GPA ifaka phakathi:

• Imithi yokuvikela ukulahleka kwamathambo okubangelwa i-prednisone
• I-Folic acid noma i-folinic acid, uma uthatha i-methotrexate
• Ama-antibiotic ukuvimbela izifo zamaphaphu

Amaqembu okusekela nabanye abaphethwe yizifo ezifanayo angasiza abantu abanalesi sifo kanye nemindeni yabo ukuthi ifunde ngalezi zifo futhi ivumelane nezinguquko ezihambisana nokwelashwa.

Ngaphandle kokwelashwa, abantu abanezinhlobo ezinzima zalesi sifo bangafa ezinyangeni ezimbalwa.

Ngokwelashwa, umbono weziguli eziningi muhle. Iningi labantu abathola i-corticosteroids neminye imithi eyehlisa ukuphendula kwamagciwane iba ngcono kakhulu. Iningi labantu abane-GPA baphathwa ngemithi eqhubekayo yokuvikela ukubuyela emuva okungenani izinyanga eziyi-12 kuye kwezingu-24.

Izinkinga zivame ukwenzeka lapho lesi sifo singelashwa. Abantu abane-GPA bahlakulela ukulimala kwezicubu emaphashini, ezindleleni zomoya, nasezinso. Ukubandakanyeka kwezinso kungaholela egazini kumchamo nasekuhlulekeni kwezinso. Isifo sezinso singakhula ngokushesha. Umsebenzi wezinso kungenzeka ungathuthuki noma ngabe isimo silawulwa yimithi.

Uma kungalashwa, ukwehluleka kwezinso futhi mhlawumbe nokufa kwenzeka ezimweni eziningi.

Ezinye izinkinga zingafaka:

• Ukuvuvukala kwamehlo
• Ukwehluleka kwamaphaphu
• Akhwehlele igazi
• I-Nasal septum perforation (imbobo ngaphakathi kwekhala)
• Imiphumela engemihle evela emithini esetshenziselwa ukwelapha lesi sifo

Shayela umhlinzeki wakho uma:

• Uba nobuhlungu besifuba nokuphefumula okuncane.
• Ukhwehlela igazi.
• Unegazi emchameni wakho.
• Unezinye izimpawu zalesi sifo.

Akukho ukuvimbela okwaziwayo.

Phambilini: I-Wegener’s granulomatosis

• I-Granulomatosis ene-polyangiitis emlenzeni
• Uhlelo lokuphefumula

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UYates M, Watts RA, Bajema IM, et al. Izincomo ze-EULAR / ERA-EDTA zokuphathwa kwe-vasculitis ehlobene ne-ANCA. [ukulungiswa okushicilelwe kuvela ku- U-Ann Rheum Dis. 2017;76(8):1480]. U-Ann Rheum Dis. 2016; 75 (9): 1583-1594. I-PMID: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.