Hauv cov lus kho mob, Goodpasture's syndrome txhais tau tias yog ib qho mob tshwj xeeb, uas yog txiav txim siab los ntawm cov kab mob autoimmune uas ua rau muaj kev puas tsuaj rau hauv qab daus daim nyias nyias ntawm alveoli ntawm lub ntsws, nrog rau lub raum glomeruli, piv txwv li ob lub cev koom nrog. cov txheej txheem pathological: lub ntsws thiab lub raum. Lub cev tsim cov tshuaj tiv thaiv rau cov kab mob hauv lub cev.
Tag nrho cov lus hais saum toj no tau ua rau hnyav dua los ntawm nephritis thiab glomerulonephritis
Qhov kev tshwm sim tseem ceeb yog rov tshwm sim dua thiab ua rau lub ntsws los ntshav ua ke nrog glomerulonephritis.
Cia peb saib seb hom kab mob Goodpasture's syndrome yog dab tsi.
keeb kwm thiab kev txheeb cais
Thawj qhov kev kho mob tshwm sim ntawm tus kab mob no tau piav qhia thiab ua haujlwm los ntawm Goodpasture xyoo 1919, yog li lub npe ntawm tus mob no. Thaum muaj tus kab mob khaub thuas kis thoob qhov txhia chaw, tus mob no tau raug cais tawm raws li ib qho kev mob hnyav ntxiv, xws li kev sib xyaw ntawm glomerulonephritis thiab pulmonary hemorrhage.
Qhov no yog ib qho tsis tshua muaj pathology - tus mobGoodpasture thiab hemoptysis, lawv muaj ntau dua rau cov tub ntxhais hluas hnub nyoog 12 txog 35 xyoo, feem ntau yog txiv neej.
Hauv cov tebchaws nyob sab Europe, qhov twg nws tshwm sim ntau zaus, qhov tshwm sim yog 1: 2 lab.
YEtiology ntawm Syndrome
Hauv cov tshuaj niaj hnub no tsis muaj ib lo lus teb hais txog etiology ntawm tus kab mob
Tsuas muaj ob peb lub tswv yim, ntawm cov hauv qab no ua rau Goodpasture's syndrome tuaj yeem paub qhov txawv:
- Kev puas tsuaj rau cov ntaub so ntswg los ntawm kev aspiration ntawm cov kuab tshuaj organic thiab cov tshuaj tsis haum hydrocarbons.
- Qee cov kws tshawb fawb xav txog qhov mob no tsis yog ib yam kab mob sib txawv, tab sis raws li kev hloov pauv ntawm idiopathic pulmonary hemosiderosis. Qhov kev xav no yog ua raws li cov pov thawj tshwm sim rau qhov muaj nyob ntawm lub xeev hloov pauv ntawm ob kab mob, ob qho tib si kho mob thiab kev txiav txim siab pathological.
- txheej txheem rau crushing pob zeb hauv urolithiasis.
- Zaj dab neeg, qee qhov HLA noob.
- Tseem muaj kev kwv yees txog tus kab mob kis kab mob, tab sis tsis muaj qhov tseeb txaus tau sau txog qhov kev kwv yees no.
Pathogenesis ntawm Goodpasture's syndrome
Cia peb xav txog qhov nthuav dav ntxiv ntawm cov yam ntxwv ntawm lub cev ntawm cov qauv ntawm cov kabmob cuam tshuam thiab cov yam ntxwv ntawm cov kab mob no.
Nrog Goodpasture's kab mob, cov alveoli thiab glomeruli ntawm ob lub raum raug cuam tshuam.
Alveoli yog pawg ua pa ua pa uas nyob rau ntawm qhov kawg ntawm qhov tsawg tshaj plawsbronchioles. Cov phab ntsa ntawm lub alveoli muaj ob txheej: ib txheej ntawm epithelium, uas muaj ncaj qha kev sib cuag nrog huab cua, thiab ib txheej ntawm endothelial hlwb, uas nyob rau ntawm phab ntsa ntawm cov ntshav capillaries. Qhov sib txawv ntawm cov khaubncaws sab nraud povtseg muaj cov membrane tshwj xeeb, hauv qab daus daim nyias nyias, uas nkag mus rau oxygen thiab carbon dioxide.
Lub raum glomeruli yog cov khoom siv hauv lub raum, lawv qhov chaw ua haujlwm tsawg tshaj plaws. Lawv muaj ib lub capsule thiab capillary network uas nyob rau hauv no capsule. Sab hauv ntawm lub capillaries muaj cov txheej txheem tshwj xeeb ntawm endothelium, thiab sab nraud, tig mus rau cov tshuaj ntsiav, sawv cev los ntawm podocytes. Nruab nrab ntawm lawv tus kheej, lawv tau sib cais los ntawm lub hauv paus hauv qab daim nyias nyias, uas muaj kev ua haujlwm dhau los - nws kis cov ntsev, dej, cov protein los ntawm cov ntshav mus rau hauv cov tshuaj ntsiav. Qhov chaw ntawm lub hauv paus membrane tsis txwv rau qhov chaw uas tau piav saum toj no. Nws kuj tseem cais lub raum tubules, uas nws txoj haujlwm yog los tso zis thawj zaug. Nws kuj tseem cais cov ntshav capillaries rau hauv cov kua dej uas nqus los ntawm nws.
Summarizing tag nrho cov saum toj no, nws yuav tsum tau muab sau tseg hais tias hauv qab daus daim nyias nyias yog ib hom tshuaj lom neeg lim uas tshem tawm carbon dioxide thiab tshuaj tsim thaum lub sij hawm lawv metabolism hauv lub cev, thiab kuj muab oxygen.
Raws li, yog tias daim nyias nyias puas lawm, tag nrho cov txheej txheem metabolic no cuam tshuam.
Antibody formation
Saum toj no hauv lub ntsiab lus, peb tau sau tseg tias kev puas tsuaj rau cov ntaub so ntswg yog vim tsim cov tshuaj tiv thaiv rau nws tus kheej (tiv thaivtshuaj). Cov txheej txheem no belongs rau qeb ntawm autoimmune. Nws hloov tawm hais tias cov tshuaj tiv thaiv tawm tsam lawv tus kheej cov ntaub so ntswg, tsim deposits ntawm lub pathological txheej, yog li ua rau nws. Qhov tshwm sim ntawm cov txheej txheem autoimmune no yog pulmonary hemorrhage thiab glomerulonephritis, cov txheej txheem ntawm lub raum glomeruli.
Txawm hais tias cov qauv ntawm vascular endothelium ntawm pulmonary capillaries yog qhov uas nws tsis tso cai rau cov tshuaj tiv thaiv nkag mus rau hauv nws, txawm li cas los xij, nyob rau hauv qee qhov tsis zoo, qhov nce hauv vascular permeability tshwm sim, vim li ntawd cov tshuaj tiv thaiv tseem nkag mus rau hauv qab daus daim nyias nyias.
yam tsis zoo
Cov yam tsis zoo ntawm Goodpasture's syndrome muaj xws li:
- nce siab ntawm oxygen hauv cov pa nqus;
- pulmonary hypertension;
- septic ntshav lesions lossis kev qaug rau lub cev;
- cov txheej txheem kis mob ntawm txoj hlab pa sab saud;
- smoking;
- aspiration ntawm roj av vapors lossis lwm hom hydrocarbon derivatives.
Dab tsi cuam tshuam feem ntau, lub raum lossis lub ntsws, tsis tau tsim. Txawm li cas los xij, qhov zaus ntawm qhov tshwm sim ntawm thawj qhov txhab ntawm lub ntsws yog siab dua li ntawm lub raum cov ntaub so ntswg.
Histological kuaj ntawm lub ntsws cov ntaub so ntswg qhia pom muaj necrotizing alveolitis. Raws li peb tau sau tseg saum toj no, qhov no yog cov ntaub so ntswg hloov pauv zoo ib yam li hauv idiopathic hemosiderosis.
Histological nta ntawm pathology
Kev kuaj mob raws sijhawm ntawm tus mobGoodpasture tseem ceeb heev.
Histological kuaj ntawm lub raum cov ntaub so ntswg qhia pom muaj nephronephritis (ua ke ntawm lipoid dystrophic degeneration ntawm ob lub raum thiab diffuse glomerulonephritis). Kev txhim kho ntawm focal intracapillary thrombotic hloov pauv thiab glomerular fibrosis kuj tau kuaj pom.
Cov tsos mob ntawm Goodpasture's syndrome yog dab tsi?
Symptomatology thiab kuaj pom
Cov no suav nrog:
- hemoptysis thiab anemia vim qhov tshwm sim ntawm pulmonary los ntshav;
- cov tsos mob ua pa luv, poob phaus, mob hauv siab;
- X-ray ntawm lub ntsws qhia kev hloov pauv ntawm tus kheej me me ntawm lub ntsws qauv hauv daim ntawv ntawm kev nplua-mesh deformity;
- cov tsos mob ntawm lub raum puas: raws li peb tau sau tseg saum toj no, lawv feem ntau koom nrog tom qab kev puas tsuaj rau lub ntsws;
- urinalysis kuaj pom muaj protein, ntshav kuj kuaj pom hauv cov zis.
- kuaj ntshav qhia txog qib siab ntawm cov khoom seem ntawm nitrogen, nce hypochromic iron deficiency anemia, tiv thaiv keeb kwm yav dhau los ntawm kev kis mob thib ob, cov ntshav suav qhia tias.
Nws tsim nyog sau cia tias ntshav pulmonary los ntshav tsis yog ib txwm tshwm sim tawm tsam keeb kwm ntawm hemoptysis, thiab qhov hnyav ntawm cov ntshav tsis nyob ntawm qhov hnyav ntawm hemoptysis. Feem ntau, los ntshav tshwm sim ntawm xob laim ceev thiab ua rau qhov tshwm sim tuag hauv ib teev xwb. Ua tsis taus pa hnyav zuj zus, pulmonary edema tshwm sim, thiab mob ntsws fulminant tshwm sim.
Clinicalduab
Tawm tsam keeb kwm yav dhau los ntawm cov yam ntxwv dav dav ntawm cov tsos mob tau piav qhia saum toj no, nws yog ib txwm ua kom paub qhov txawv ntawm peb hom kab mob:
- malignant form. Nws yog tus cwj pwm los ntawm qhov muaj qhov tshwm sim ntawm kev mob ntsws thiab kev loj hlob sai ntawm glomerulonephritis.
- Kev txhim kho qeeb ntawm kev hloov pauv hauv lub ntsws thiab lub raum cov ntaub so ntswg.
- Kev nce qib glomerulonephritis uas ua rau muaj kev hloov pauv hauv lub ntsws hauv lub sijhawm luv tshaj plaws ua rau mob raum tsis ua haujlwm.
Nyob rau me nyuam
Vim li cas tus menyuam thiaj li tsim muaj kab mob? Yog vim li cas rau qhov no yog lub hereditary predisposition ntawm tus me nyuam, uas yuav kis tau los ntawm nws niam nws txiv. Feem ntau, tus kab mob no tshwm sim hauv cov menyuam mos uas nws niam thaum lub sij hawm yug me nyuam coj txoj kev ua neej tsis zoo thiab haus luam yeeb. Yog li ntawd, tus me nyuam hauv txoj kev loj hlob tsis tau txais cov pa oxygen tsim nyog rau lub cev, thiab nws lub ntsws tsuas yog hloov mus rau hauv lub ntsws ntawm tus neeg haus luam yeeb. Tsis tas li ntawd, cov xwm txheej ntxiv tuaj yeem cuam tshuam tsis zoo rau tus menyuam noj qab haus huv, xws li, piv txwv li, muaj cov kab mob sib kis uas cuam tshuam rau txoj kev ua pa, nrog rau kev nqus pa ntawm hydrocarbon vapors.
Goodpasture's syndrome nyob rau hauv cov menyuam yaus feem ntau pib mob heev, nrog rau qhov kub thiab txias kom muaj nuj nqis, hemoptysis, pulmonary hemorrhage, mob siab ua pa tshwm sim. Kev mloog qhia tau hais tias lub suab ntub dej hauv lub ntsws. Glomerulonephritis tshwm sim feem ntau, txawm tias tom qab, tab sis sai txaus. Raws li tau hais los saum no, kev txhim kho ntawm lub raum tsis ua haujlwm ua raws yuav luag tam sim ntawd. Ib tug yam ntxwv immunological marker ntawm systemickab mob yog muaj cov tshuaj tiv thaiv rau hauv qab daus daim nyias nyias ntawm lub raum.
Nws yog ib qho tsim nyog los kho cov kab mob no, tshwj xeeb tshaj yog nyob rau hauv cov menyuam yaus, nyob rau hauv kev saib xyuas nruj ntawm cov kws tshaj lij, thaum ua kom pom tseeb ntawm txoj hlab ntsws tseem ceeb heev.
Vim tias Goodpasture's syndrome tau tsim muaj kev mob siab rau ob qho tib si hauv cov menyuam yaus thiab cov neeg laus, thaum tsis muaj kev kuaj mob kom raug, nrog rau kev kho kom zoo, tus kab mob tuaj yeem ua rau muaj kev cuam tshuam ntau dua. Ntxiv mus, hauv kev kho mob, muaj cov neeg tuag.
Huab cua
Kev ntsuas ntawm pathology, hmoov tsis, yog qhov tsis zoo. Tus neeg mob tuag qhov nruab nrab hauv ib xyoos. Raws li peb tau sau tseg saum toj no, kuj tseem muaj cov qauv ntawm cov kab mob fulminant, thaum hais ib lub lim tiam dhau los ntawm thawj cov tsos mob ntawm tus kab mob ua npaws mus rau qhov ua rau tuag taus.
Mechanism of disease progression
Goodpasture's syndrome yog ib yam kab mob autoimmune, uas yog, tshwm sim los ntawm kev tsim cov tshuaj tiv thaiv rau ib tus kheej lub hlwb. Lub glomerular hauv qab daim nyias nyias yog qhov uas cov tshuaj tiv thaiv no tau tsim. Cov tshuaj tiv thaiv no khi rau ib qho tshwj xeeb hauv plaub hom collagen.
Nws yog ib feem ntawm plaub hom collagen uas yog lub hom phiaj ntawm cov tshuaj tiv thaiv. Qhov no ntawm collagen hom 4 yog hu ua Goodpasture antigen.
Hauv cov neeg noj qab haus huv, cov tshuaj tiv thaiv no tsis yog qhov ua rau cov kab mob pathological. Tus kab mob no cuam tshuam rau lub ntsws thiab lub raum cov ntaub so ntswg, vim hais tias nws yog nyob rau hauv cov ntaub so ntswg uascoob tus antigens ntawm hom no.
Thaum ib qho tshuaj tiv thaiv kab mob khi rau Goodpasture antigen, cov txheej txheem ntxiv tau tshwm sim. Cov no yog cov tshuaj tiv thaiv kab mob, los yog lawv hom tshwj xeeb. Cov ntawv cog lus tsim no yog qhov ua rau muaj kev cuam tshuam rau kev sib txuas ntawm cov kab mob pathological. Nyob rau hauv kev tsom xam ntawm kev sib cuag ntawm cov tshuaj tiv thaiv thiab antigens, kev sib txuas tshwm sim nrog leukocytes.
Txhua yam mus rau qhov tseeb tias leukocytes nquag tawm tsam cov ntaub so ntswg cuam tshuam, yog li rhuav tshem lawv. Lub cev tiv thaiv kab mob rau cov txheej txheem no yog qhov nce ntxiv ntawm cov cell epithelial. Lawv qhov tseem ceeb deposition tshwm sim nyob rau saum npoo ntawm lub hauv paus membrane. Lub cev pib poob nws txoj haujlwm sai, tsis tuaj yeem tiv nrog lub nra, cov khoom pov tseg sai sai hauv lub cev.
Mechanisms ntawm pathological hloov hauv lub raum thiab lub ntsws muaj ib qho kev kawm zoo ib yam.
Kev kho mob ntawm Goodpasture's syndrome
Tsuas muaj txoj hauv kev los ua kom tus kab mob qeeb qeeb kom thiaj li ua rau tus neeg mob lub neej ntev. Rau lub hom phiaj no, corticosteroids thiab immunosuppressants raug sau tseg, hemodialysis yog ua, tom qab ntawd, yog tias tsim nyog, nephrectomy thiab hloov lub raum, uas tso cai rau tshem tawm cov tshuaj tiv thaiv antigenic. Plasmapheresis tshem tawm cov tshuaj tiv thaiv kab mob.
Nws tau pom tias cov tshuaj tiv thaiv kab mob hauv qab daus tsis raug kuaj pom hauv cov ntshav ntshav nyob rau nruab nrab rau lub hlis tom qab pib tus kabmob. Tias yog vim li cas thiaj li muaj kev xav tias cov kev ntsuas mob hnyav uas tau hais los saum toj no tuaj yeem ua rau tus neeg mob lub neej ntev mus txog. Kev txiav tawm ntawm cov txheej txheem pathological autoimmune.
ntsuas cov tsos mob suav nrog kev hloov ntshav thiab ntshav tsis muaj hlau tsis txaus.
Nyob hauv Goodpasture's syndrome, cov lus qhia kho mob yuav tsum ua raws li nruj me ntsis.
