Wegener's granulomatosis yog ib qho mob ntawm cov phab ntsa vascular, uas muaj tus cwj pwm autoimmune granulomatous. Tus kab mob no loj heev vim hais tias nws cuam tshuam rau sab sauv ua pa, ntsws, ob lub raum, ob lub qhov muag thiab lwm yam kabmob ntxiv rau capillaries, hlab ntsha, venules thiab arterioles.
Vim li cas thiaj tshwm sim? Cov tsos mob qhia nws txoj kev loj hlob li cas? Kev kuaj mob ua li cas? Dab tsi yog xav tau rau kev kho mob? Tam sim no cov lus nug no thiab ntau lwm cov lus nug yuav tsum tau teb.
Tus yam ntxwv ntawm tus kab mob
Wegener's granulomatosis yog ib yam kab mob hnyav, kis tau sai. Yog tias koj tsis saib xyuas cov tsos mob hauv lub sijhawm thiab tsis pib kho, ces kev tuag yuav tshwm sim hauv 6-12 lub hlis.
Hmoov tsis zoo, etiology tseem tsis tau tsim. Txawm li cas los xij, raws li kev txheeb cais, tus kab mob no feem ntau cuam tshuam rau cov neeg uas yav tas los raug kev txom nyem los ntawm kev kis tus kab mob ua pa. Cov kws kho mob, txawm li cas los xij, tsis suav nrog kev sib txuasgranulomatosis nrog cov kab mob tsis zoo thiab cov kab mob cab.
Nws yog ib qho tseem ceeb uas yuav tsum nco ntsoov tias lub cev tiv thaiv kab mob yuav luag ib txwm pom hauv cov ntshav ntawm cov neeg mob uas muaj qhov kev kuaj mob no, tab sis lawv lub luag haujlwm hauv cov kab mob tseem tsis tau muaj pov thawj. Ib yam li muaj cov tshuaj tiv thaiv kab mob rau cov cytoplasm, neutrophils thiab antigens DR2, HLA B7 thiab B8. Tab sis muaj ib qho kev xav tias tag nrho cov teeb meem no yog catalysts rau kev loj hlob ntawm Wegener's granulomatosis.
Qhov laj thawj kuj yuav ua rau lub cev muaj zog ntau ntxiv lossis tiv thaiv tsis muaj zog.
Symptoms
Tus kab mob no nrog rau daim duab qhia tau hais tseg. Cov tsos mob ntawm Wegener's granulomatosis yog:
- qaug zog thiab qaug zog.
- kab mob ua pa sab sauv.
- Rhinitis nrog purulent nasal paug.
- Saddle qhov ntswg deformity.
- tawm hws ntau dhau.
- Ulceration ntawm mucous daim nyias nyias, uas tuaj yeem ncav cuag perforation ntawm qhov ntswg septum.
- poob phaus tsis tsim nyog.
- mob nqaij vim yog cell hypertonicity (myalgia).
- Pom kab mob ntawm pob qij txha (mob caj dab).
Cov tsos mob ntawm Wegener's granulomatosis tshwm sim hauv yuav luag txhua kis. Qee tus kuj muaj cov tsos mob hauv qab no:
- Kev hloov pauv hauv lub ntsws ntawm lub ntsws.
- hnoos nrog hnoos qeev (xws li pleurisy).
- Kev txhim kho ntawm glomerulonephritis.
- mob raum tsis ua haujlwm.
- Hemorrhagic rash.
- Kev txhim kho ntawm cov kab mob episcleritis (tshwj xeeb hauv kev mob ntawm sclera).
- Kev tsim ntawm exophthalmos thiab orbital granulomas.
- Teeb meem nrog lub paj hlwb.
Cov mob hnyav tuaj yeem nrog kev puas tsuaj rau cov hlab ntsha thiab myocardium.
Localized form
Hom Wegener's granulomatosis no yog tus cwj pwm los ntawm cov kab mob ntawm lub ntsws sab sauv. Yog li, feem ntau cov tsos mob yog:
- Kev tsim cov ntshav txhaws hauv qhov ntswg.
- ua pa nyuaj.
Yog tus neeg mob tau kuaj pom tias muaj kev puas tsuaj loj hauv ib cheeb tsam ntawm tus kab mob, ces nws yuav tau mus phais.
Indications rau kev phais
Feem ntau lawv yog:
- Kev puas tsuaj ntawm qhov ntswg qhov ntswg. Hauv qhov no, kev phais yas ntawm lub qhov ntswg nraub qaum yog ua tiav.
- Stenosis nrog kev hem thawj ntawm kev ua pa. Txhaum lub tracheostomy (duab cua tshuab).
- Kev hem thawj ntawm qhov muag tsis pom. Cov granuloma raug kho dua.
- Terminal raum tsis ua haujlwm. Hauv qhov xwm txheej no, kev hloov lub raum tau qhia.
Nws yog ib qho tseem ceeb kom paub meej tias nrog Wegener's granulomatosis, cov lus pom zoo rau kev ua haujlwm yog muab tso rau ntawm tus kws kho mob nkaus xwb yog tias tus neeg mob lub neej muaj kev phom sij tiag tiag lossiskev tsis taus.
daim ntawv ntawm tus kab mob
Nws yog tus yam ntxwv los ntawm ntau yam kev tshwm sim. Cov no suav nrog:
- Hemorrhages.
- Ib paroxysmal hacking hnoos, nrog los ntawm ntshav-purulent hnoos qeev.
- Fever.
- mob pob qij txha.
- Abscess pneumonia.
- Lub raum thiab lub plawv tsis ua haujlwm.
- polymorphic rash.
Wegener's granulomatosis ntawm daim ntawv dav dav yog kho siv cov txheej txheem ntawm kev kho lub cev tsis zoo. Cov no suav nrog:
- Cryoapheresis. Cov tshuaj pathological raug xaiv tshem tawm ntawm cov ntshav plasma. Tseem muaj kev sib txuas. Qhov txawv ntawm plasma pom yog tias thaum lub sij hawm cryoapheresis, lub plasma txias.
- Plasmapheresis. Cov txheej txheem no suav nrog kev noj ntshav, tom qab ntawd ntxuav nws thiab rov qab mus rau cov hlab ntsha.
- Cascade plasma pom. Ib txoj kev siv thev naus laus zis ib nrab xaiv uas tso cai rau kev xaiv tshem tawm cov kab mob thiab cov kab mob los ntawm cov ntshav plasma. Ib lub tshuab lim ntshav zoo tshaj plaws hauv ntiaj teb.
- Tshuaj kho mob rau lub cev. Hemocorrection, vim hais tias cov tshuaj xa ncaj qha mus rau thaj tsam ntawm cov txheej txheem pathological. Lub peculiarity ntawm txoj kev yog nyob rau hauv lub point nyhuv. Feem ntau siv rau hauv kev kho mob ntawm Wegener's granulomatosis ntawm qhov ntswg. Siv txoj kev tso cai rau koj kom tsis txhob muaj qhov tsis zoo ntawm cov tshuaj hauv lub cev tag nrho.
Thiab tau kawgKev kho tshuaj pharmacological yog qhov yuav tsum tau ua. Cov tshuaj twg uas feem ntau raug sau rau ob hom ntawm Wegener's granulomatosis tau piav qhia hauv qab no.
Diagnosis
Nws yuav tsum tau hais txog nws qhov kev siv. Yog tias ib tug neeg pom cov tsos mob ntawm Wegener's granulomatosis (daim duab ntawm qhov tshwm sim ntawm tus kab mob tsis tau nthuav tawm vim yog kev coj ncaj ncees), ces nws yuav tsum mus ntsib kws kho mob rheumatologist.
Kev kuaj tus kab mob no yog ib txoj haujlwm nyuaj. Nws yuav tsum txawv ntawm sarcoidosis, malignant hlav, uveitis, sinusitis, otitis media, ntshav thiab raum kab mob, tuberculosis thiab lwm yam kab mob autoimmune. Thawj theem suav nrog cov kev tshawb fawb hauv qab no:
- Kev tso zis thiab ntshav rau kev tshuaj ntsuam xyuas. Cov txiaj ntsig tuaj yeem qhia tias muaj ntshav qab zib hauv ib tus neeg, cov txheej txheem inflammatory, pab kom paub tseeb tias qhov ntom ntawm cov zis thiab cov ntsiab lus ntawm cov ntshav thiab cov protein nyob hauv nws hloov. Tsis tas li ntawd, txoj kev tshawb no nthuav tawm qhov nrawm ntawm erythrocyte sedimentation tus nqi, normochromic anemia, thrombocytosis, nrog rau microhematuria thiab proteinuria.
- ntshav pub dawb rau kev tshuaj xyuas biochemical. Cov txiaj ntsig yuav qhia tau tias lub siab thiab lub raum puas. Nws tseem tuaj yeem kuaj pom qhov nce hauv γ-globulin, urea, creatinine, haptoglobin, seromucoid thiab fibrin.
- Ultrasound ntawm lub raum thiab lub plab plab. Kev kuaj ultrasound yuav pab txheeb xyuas qhov mob thiab txo cov ntshav ntws.
- kuaj urological (scintigraphy, excretory urography). Cov kev tshawb fawb no yuav pab txiav txim siab qhov poob qis hauv kev ua haujlwmlub raum.
- X-ray ntawm lub ntsws. Pab kom txiav txim siab muaj cov infiltrates hauv lawv cov ntaub so ntswg, nrog rau txhawm rau txheeb xyuas pleural exudate thiab cov kab noj hniav lwj.
- Bronchoscopy nrog biopsy ntawm cov mucous daim nyias nyias ntawm lub siab ua pa ib ntsuj av. Qhov no yuav pab txheeb xyuas cov cim morphological ntawm tus kab mob.
Ntxiv rau qhov saum toj no, ua ib feem ntawm kev kuaj mob ntawm Wegener's granulomatosis, nws yuav tsum tau sab laj nrog cov kws kho mob tshwj xeeb - pulmonologist, otolaryngologist, ophthalmologist, nephrologist, urologist thiab dermatologist. Lawv, tom qab kuaj thiab nug tus neeg mob, yuav tsis suav nrog lwm yam kab mob zoo sib xws.
Kev kho mob
Kev kho rau Wegener's granulomatosis yog kws kho mob tau sau tseg tom qab kuaj tiav.
Raws li txoj cai, cov tshuaj "Cyclophosphamide" raug sau tseg. Nws yog ib qho tshuaj cytostatic chemotherapeutic uas muaj alkylating hom kev ua. Nws muaj qhov dav dav ntawm kev ua haujlwm antitumor. Tsis tas li ntawd, cov tshuaj muaj ib tug pronounced immunosuppressive nyhuv. Noj cov tshuaj no intravenously, noj txhua hnub yog 150 mg ib hnub twg.
Cov tshuaj yog ua ke nrog "Prednisolone", cov tshuaj hluavtaws glucocorticoid, qhov kev txiav txim ntawm lub hom phiaj ntawm kev ua haujlwm ntawm cov ntaub so ntswg macrophages thiab leukocytes. Nws kuj stabilizes lysosomal daim nyias nyias, cuam tshuam lub peev xwm ntawm macrophages rau phagocytosis thiab tiv thaiv leukocytes los ntawm kev nkag mus rau qhov chaw mob. Kev noj tshuaj txhua hnub ntawm "Prednisolone" yog 60 mg. Raws li qhov xwm txheej zoo dua, tus nqi ntawm cov tshuaj tau muabtxo.
Kev kho mob ntawm Wegener's granulomatosis no yog siv rau hauv cov kab mob hauv lub cev, thaum tus kab mob npog daim tawv nqaij, pob qij txha, ob lub raum thiab qhov muag.
Kev kho tshuaj siv sijhawm ntev. Yog li, piv txwv li, "Cyclophosphamide" yuav tsum tau noj rau lwm 1 xyoos tom qab tus kws kho mob kuaj xyuas cov theem ntawm kev ua tiav ntawm cov tsos mob hnyav. Tau kawg, koob tshuaj txhua hnub yuav tsum tau maj mam txo.
Kev txhaj tshuaj tiv thaiv kab mob
Yog tsis muaj cov tshuaj no nrog Wegener's granulomatosis (daim duab ntawm granulomas nyob rau hauv lub tshuab ntsuas pom saum toj no). Vim hais tias tus kab mob no yog autoimmune nyob rau hauv cov xwm, thiab noj tshuaj los suppress lub tiv thaiv kab mob yog yuav tsum tau.
Ob cov tshuaj teev saum toj no kuj yog cov tshuaj tiv thaiv kab mob, thiab zoo tshaj plaws. Tab sis muaj lwm txoj hauv kev:
- Methotrexate. Ib qho tshuaj tiv thaiv kab mob cuam tshuam rau cov tshuaj tiv thaiv metabolite. Inhibits lub synthesis ntawm thymidylate thiab purine nucleotides. Tshwj xeeb tshaj yog tawm tsam cov hlwb loj hlob sai, nws kuj muaj cov tshuaj tiv thaiv kab mob.
- Y "Fluorouracil". Nws qhov kev txiav txim yog txhawm rau thaiv DNA synthesis thiab tsim cov txheej txheem tsis zoo RNA. Yog li cov txheej txheem ntawm kev faib cell yog inhibited.
- "Dexamethasone". Txo cov concentration ntawm proteolytic enzymes, txo capillary permeability, inhibits collagen tsim thiab fibroblast kev ua.
- "Azathioprine". Cov qauv analogue ntawm adenine, guanine thiab hypoxanthine, uas ua rau nucleic acids. Cov tshuaj thaiv cov ntaub so ntswg proliferation thiab cell faib, thiab kuj cuam tshuamantibody synthesis.
- YChlorbutin. Nws muaj cov nyhuv alkylating. Cov tshuaj muaj cytostatic cov cuab yeej, yog li nws kuj cuam tshuam DNA replication. Nws muaj qhov tshwj xeeb muaj zog ntawm cov ntaub so ntswg lymphoid.
Cov tshuaj tau teev tseg txo cov qib ntawm kev tiv thaiv kab mob uas tau nce nrog Wegener's granulomatosis, cuam tshuam rau lub cev "raging". Hauv lwm lo lus, txwv nws cov tshuaj tiv thaiv.
Hmoov tsis zoo, qhov kev mob tshwm sim yog qhov txo qis hauv tag nrho lub cev tsis kam mus rau ntau yam kab mob thiab cov kab mob metabolic, tab sis yog tias tsis noj cov tshuaj no, tus kab mob tsis tuaj yeem tiv nrog.
Yuav ua li cas txog koob tshuaj thiab hom kev tswj hwm? Qhov no yog txiav txim los ntawm ib tug rheumatologist. Rau qee tus neeg mob, piv txwv li, kev kho lub plawv yog tsim nyog - kev tswj hwm ntawm cov tshuaj hauv koob tshuaj loj 1-2 zaug hauv ib lub lis piam.
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Thaum tus neeg mob zoo lawm, txhua lub zog raug coj los tswj nws txoj kev zam txim. Ua li no, koj yuav tsum tau mus txuas ntxiv mus rau kev kho mob immunosuppressive, txwv tsis pub tus kab mob yuav nce ntxiv. Tab sis dab tsi tshuaj noj, thiab nyob rau hauv dab tsi kom muaj nuj nqis - qhov no yog txiav txim los ntawm tus kws kho mob.
Kev zam txim tuaj yeem ntev. Tab sis tus neeg mob yuav tsum tau npaj rau relapses. Thaum tus kab mob no zuj zus lawm, nws yuav tsum tau nce qib ntawm cov tshuaj uas muaj cov tshuaj hormones los yog rov qab mus rau cov qauv uas tau muab rau thaum pib kho.
Ntau tus neeg, los ntawm txoj kev, xav paub seb puas muaj cov tshuaj pej xeem zoo uas tuaj yeem pab daws qhov nokab mob? Cov lus teb yog pom tseeb. Txawm hais tias tsis muaj kev npaj tshuaj pharmacological, kev siv uas tuaj yeem ua rau rov ua tiav, tom qab ntawd kev kho tshuaj ntsuab yuav tsis pab. Infusions thiab decoctions tsuas yog txo cov tsos mob tsis zoo. Tab sis txawm nyob rau hauv cov ntaub ntawv no, lawv yuav tsum tau tham nrog koj tus kws kho mob ua ntej.
Kev Teeb Meem thiab Kev Ntsuas
Yog tias kev kho tus kab mob tau pib raws sijhawm, ces txoj hauv kev ntawm kev tshem tawm yog siab heev. Nrog kev kho kom raug, nws tshwm sim hauv yuav luag txhua tus neeg mob.
Tab sis kev kis tus kab mob ua rau muaj qhov tshwm sim loj, suav nrog:
- Kev puas tsuaj ntawm lub ntsej muag pob txha.
- raum tsis ua haujlwm.
- mob conjunctivitis.
- Ua pa tsis ua haujlwm.
- Kev txhim kho necrotic granulomas hauv lub ntsws cov ntaub so ntswg.
- Ntsuab ntiv tes.
- Kev txhim kho ntawm Pneumocystis pneumonia.
- Yuav pob ntseg vim mob otitis.
- Gangrenous thiab trophic hloov hauv ko taw.
- Kev nkag mus ntawm cov kab mob thib ob ntawm keeb kwm ntawm kev tiv thaiv tsis muaj zog.
Yog ib tug neeg tsis quav ntsej cov tsos mob thiab tsis tig mus rau tus kws kho mob tshwj xeeb, ces qhov txiaj ntsig zoo yuav tsis suav nrog. Kwv yees li 93% ntawm cov neeg mob tuag ntawm 5 lub hlis thiab 2 xyoos.
Nws tseem ceeb heev uas yuav tsum nco ntsoov tias daim ntawv hauv zos ntawm granulomatosis yog qhov zoo. Thaum ua cov kev kho mob zoo, hauv 90% ntawm cov neeg mob qhov mob zoo zuj zus tuaj, thiab hauv 75% kev tshem tawm tshwm sim. Tab sis, hmoov tsis, rau 50% nws tsis kav ntev,kwv yees li rau lub hlis, tom qab ntawd muaj qhov exacerbation thiab tag nrho cov tsos mob rov qab.
Yog li ntawd, txhawm rau kom tsis txhob muaj teeb meem thiab mob hnyav, nws yog qhov yuav tsum tau ua kom muaj zog ntxiv rau koj txoj kev tiv thaiv kab mob, kho cov kab mob sib kis raws sijhawm, mus ntsib kws kho mob rheumatologist tsis tu ncua thiab ua raws li tag nrho nws cov lus pom zoo yam tsis muaj kev zam.
