Wegener's granulomatosis yog ib hom kab mob autoimmune uas hais txog cov mob hnyav thiab mob hnyav sai heev. Kev mob vasculitis thiab Wegener's granulomatosis yog zoo li cov ntsig txog pathologies, vim tias cov tshuaj tiv thaiv (antineutrophil cytoplasmic) yog tsim nyob hauv granulomatosis, uas yog tus cwj pwm ntawm ANCA-vas vasitis.
Ua rau Wegener's granulomatosis
Wegener's granulomatosis hais txog kev kho mob autoimmune, yog li yuav muaj qhov zoo tshaj. Qhov tseeb, granulomatosis yog ib qho kev tiv thaiv kabmob tsis zoo. Yog li, cov cim ntawm tus kab mob no yog antigens - HLA B B1, B_8, 〖DR〗 _2, 〖DQ〗 _w7.
Lub luag haujlwm ntawm cov kab mob pathogens tseem ua si los ntawm antineutrophil cytoplasmic antibodies reacting proteinase-3.
Wegener's granulomatosis - cov tsos mob
Cov tsos mob ntawm tus kab mob granulomatosis feem ntau tshwm sim thaum muaj hnub nyoog 40 xyoo, thaum qhov tseem ceeb ntawm tus poj niam los txiv neej tsis tseem ceeb.
Granulomatosis - ib qho kev mob ntawm cov phab ntsa ntawm me me thiab medium vessels: venules, capillaries, arteries thiab arterioles. Hauv txoj kev sib tw, txoj hlab ntsws qis, ob lub raum, ob lub qhov muag, lub ntsws thiab lwm yam kabmob yog kev koom tes.
Cov tsos mob muaj raws li nram no:
- kev hnyav - kev poob qis, kev qaug zog, phev siab, mob plawv, kub cev, mob qis dua, mob caj dab;
- lub paj hlwb - asymmetric polyneuropathy;
- ob lub qhov muag - ischemia ntawm lub paj hlwb, dig muag, episcleritis nrog orbital granuloma thiab exophthalmos;
- daim tawv nqaij - pob ua pob ua ke nrog cov yeeb yam necrotic;
- ob lub raum - glomerulonephritis , uas tuaj yeem coj ceev ceev;
- lub ntsws - cov qhov txhab tsim tau hauv 75% ntawm cov neeg mob nyob rau hauv daim ntawv ntawm infiltrates, uas tsim kab noj hniav thaum lub sijhawm hniav lwj; nrog rau no yuav ua tau cai pleauty, hnoos.
- kab mob ntsws - yog cuam tshuam rau hauv 90% ntawm cov neeg mob hauv daim ntawv ntawm rhinitis nrog purulent-haemorrhagic secretion thiab ulceration; perforation ntawm qhov ntswg septum ua rau eeb deformation; Tsis tas li ntawd, qhov kev mob tshwm sim muaj feem xyuam rau cov kab xo, pob ntseg, lub cev, cov hlab ntsha hauv vaj hauv tsev, uas nyob rau hauv qhov txheej txheem necrotic.
Wegener's granulomatosis kuj muaj ob hom:
- localized - nws muaj feem xyuam rau feem ntau ntawm cov pa nqaj qaum;
- generalized - nrog nws ntau lub nruab nrog cev muaj feem cuam tshuam, thiab qhov no yog nrog nws kub cev, mob leeg leeg, hnoos thiab lub plawv tsis ua hauj lwm.
Kev kuaj ntawm Wegener's granulomatosis
Tus mob no yog tsim los ntawm ib tug kws kho mob raws li ntau cov ntaub ntawv:
- kev ntsuam xyuas ntshav - biochemical thiab dav, uas tsis tuaj yeem tshawb xyuas tau tus kab mob granulomatosis, tab sis pab txiav tawm lwm cov kab mob;
- zis tsom xam - nws muaj peev xwm los tshawb xyuas los yog tshem tawm lub raum puas tsuaj (daim ntawv ntawm tus kab mob tau txiav txim siab);
- Xoo hluav taws xob ntawm paranasal sinus thiab thorax (computed tomography muab cov ncauj lus qhia ntau dua li ib qho tau los ntawm X-ray);
- txoj kev lis dej num - tsuas yog txoj kev uas tseeb los yog tsis pom muaj cov mob granulomatosis; cov kws kho mob siv cov ntaub so ntswg los ntawm lub qhov ntswg lossis lub qa, thiab qee zaum, lub raum thiab lub ntsws.
Kev kho mob ntawm Wegener tus granulomatosis
Kev kho mob ntawm tus kab mob yog nqa tawm, feem ntau, nrog kev koom tes ntawm corticosteroids thiab cytostatics, uas txo cov kev ua si
Nrog rau lub raum loj heev, qee zaum, tus neeg mob xav tau kev hloov ntshav.
Wegener lub granulomatosis - prognosis
Yog tias kev kho tsis tau pib rau lub caij nyoog, ces qhov kev tsim nyog yuav tsis tuaj yeem muaj tseeb hauv 6-12 lub hlis, thiab qhov nruab nrab ntawm lub neej tsis pub tshaj 5 hlis.
Nyob rau hauv qhov teeb meem ntawm kev kho, remission kav txog 4 xyoos, qee lub sij hawm 10 xyoo. Ib qho kev kho tag nrho ntawm tam sim no theem ntawm txoj kev loj hlob ntawm cov tshuaj yog tsis yooj yim sua.