Koj puas muaj kev o ntawm koj lub cev? Cia peb kawm txog tus mob tshiab no hu ua VEXAS Syndrome.

Puas muaj tej lub sij hawm koj xav tias muaj tej yam txawv txawv los ntawm sab hauv koj lub cev, xws li o? Puas muaj tej lub sij hawm koj kub taub hau, mob pob qij txha, teeb meem ntawm daim tawv nqaij, thiab lwm yam? Txawm hais tias tej no zoo li tsis muaj feem cuam tshuam, tej zaum qhov ua rau tag nrho cov no yuav yog tib yam mob uas tsis tshua muaj. Ib qho mob zoo li no hu ua VEXAS Syndrome . Cia peb tham me ntsis txog qhov no hnub no, vim nws yog ib qho tseem ceeb heev uas yuav tsum paub txog qhov no.

VEXAS Syndrome yog dab tsi? Hais yooj yim xwb...

Hais yooj yim xwb, VEXAS Syndrome yog ib yam mob autoimmune uas tsis tshua muaj . Tam sim no tej zaum koj yuav xav paub tias tus mob autoimmune no yog dab tsi. Xav txog seb, peb lub cev muaj lub cev tiv thaiv kab mob. Nws zoo li ib pab tub rog uas tiv thaiv peb lub teb chaws. Txoj hauj lwm ntawm lub cev no yog los tiv thaiv peb los ntawm kev tawm tsam cov kab mob thiab cov kab mob uas los ntawm sab nraud. Txawm li cas los xij, qee zaum tus neeg tiv thaiv no, lub cev tiv thaiv kab mob, pib ua yuam kev tawm tsam cov hlwb thiab cov nqaij ntawm peb lub cev . Zoo li peb tsis tuaj yeem qhia leej twg yog peb tus kheej thiab leej twg yog tus yeeb ncuab. Qhov ntawd yog qhov peb hu ua tus mob autoimmune.

Qhov ntawd yog qhov tshwm sim rau ib tus neeg uas muaj VEXAS Syndrome. Lub cev tiv thaiv kab mob tawm tsam ntau qhov ntawm lub cev, ua rau o thiab o . Zoo li muaj hluav taws me me nyob hauv lub cev tas li.

VEXAS Syndrome tuaj yeem cuam tshuam rau cov hauv qab no ntawm lub cev:

• Rau koj cov ntshav
• Mus rau cov pob txha
• Rau cov hlab ntsha
• Rau koj daim tawv nqaij
• Pob txha mos (tshwj xeeb tshaj yog hauv pob ntseg thiab qhov ntswg)
• Rau cov pob qij txha
• Mus rau lub ntsws
• Rau lub qhov muag
• Cov npe ntawm cov noob qes hauv cov txiv neej

Xav txog seb qhov no yuav ua rau muaj teeb meem ntau npaum li cas thaum muaj ntau qhov chaw raug cuam tshuam ib zaug. Qhov ua rau muaj tus kab mob no yog kev hloov pauv caj ces . Yog xav kom meej, kev hloov pauv hauv ib lub noob hu ua UBA1 noob . Lub noob UBA1 no tsim cov enzyme E1 ubiquitin-activating, lossis E1 enzyme luv luv. Lub luag haujlwm ntawm cov enzyme no yog los ntxuav cov khoom pov tseg xws li cov protein tsis tsim nyog uas sib sau ua ke hauv peb lub hlwb thiab pab kho qhov puas tsuaj rau cov hlwb. Nws zoo li ib tug neeg tshem tawm cov khib nyiab hauv peb lub tsev. Tab sis thaum koj muaj VEXAS Syndrome, cov enzyme E1 uas tsim los ntawm UBA1 noob tsis ua haujlwm zoo. Yuav ua li cas tom qab ntawd? Cov khib nyiab sib sau ua ke hauv cov hlwb.

Qhov tseem ceeb tshaj plaws yog tias yog tias tus mob no tsis kho kom zoo, qee zaum nws tuaj yeem ua rau tuag taus.Yog li ntawd, nws yog ib qho tseem ceeb uas koj yuav tsum nrhiav kev pab tswv yim los ntawm kws kho mob yog tias koj muaj cov tsos mob. Cov kws kho mob yuav muab kev kho mob tsim nyog los tswj koj cov tsos mob.

Lub npe VEXAS txhais li cas?

Lub npe VEXAS yog kev sib xyaw ua ke ntawm cov tsiaj ntawv thawj zaug ntawm ntau lo lus uas pab txheeb xyuas tus kab mob no. Cia peb saib lawv yog dab tsi:

• V - Vacuoles: Cov no yog cov puag ncig, khoob qhov chaw uas tsim nyob rau hauv cov hlwb tsis zoo. Cov vacuoles no tuaj yeem pom hauv cov hlwb pob txha ntawm cov neeg uas muaj VEXAS Syndrome.
• E - E1 enzyme: Raws li peb tau hais ua ntej, qhov no yog E1 enzyme uas tsis ua haujlwm zoo vim muaj kev hloov pauv hauv UBA1 gene.
• X - X-txuas: Koj paub tias peb cov poj niam txiv neej yog txiav txim siab los ntawm ob lub chromosomes. Cov poj niam muaj XX chromosomes thiab cov txiv neej muaj XY chromosomes. Lub noob caj noob ces UBA1 uas hloov pauv uas ua rau VEXAS Syndrome nyob ntawm X chromosome.
• A - Autoinflammation: Qhov no yog lub npe kho mob rau kev o uas tshwm sim thaum lub cev tiv thaiv kab mob tawm tsam nws lub cev.
• S - Somatic: Qhov kev hloov pauv caj ces uas ua rau muaj VEXAS Syndrome yog ib hom kev hloov pauv hu ua "somatic." Qhov no txhais tau tias nws tshwm sim yam tsis tau npaj tseg thiab tsis tau txais los ntawm niam txiv. Qhov no txhais tau tias koj tsis tas yuav txhawj txog koj cov menyuam yuav tau txais nws tsuas yog vim koj muaj nws.

Koj puas nkag siab tias lub npe VEXAS tau los li cas? Txhua tsab ntawv no qhia txog ib qho tseem ceeb txog tus kab mob.

VEXAS Syndrome no muaj ntau npaum li cas?

Qhov no yog ib qho mob tsis tshua muaj . Cov kws tshaj lij hais tias txawm tias nyob hauv lub tebchaws zoo li Asmeskas, tus kab mob no cuam tshuam txog li ib ntawm 13,000 tus neeg. Txawm hais tias cov ntaub ntawv hauv Sri Lanka tsis yog qhov tseeb, nws yog qhov tseeb tias qhov no yog ib qho mob uas tsis tshua muaj.

Cov tsos mob ntawm VEXAS Syndrome yog dab tsi?

Cov tsos mob tseem ceeb ntawm tus kab mob no yog o . Yog li, lwm cov tsos mob nyob ntawm seb qhov twg hauv lub cev o. Saib seb koj puas muaj ib qho ntawm cov tsos mob no:

• Kuv feem ntau kub taub hau.
• Cov pa oxygen tsawg hauv cov ntshav (hypoxemia) .
• Ntau yam pob khaus tawv nqaij thiab eczema.
• Lub cev o.
• Mob pob qij txha.
• Hnoos.
• Ua pa nyuaj (ua tsis taus pa).
• Qhov muag liab.
• Mob taub hau.
• Kev o ntawm cov noob qes rau cov txiv neej (orchitis).

Yog tias muaj ib lossis ntau yam ntawm cov tsos mob no tseem muaj, nws yog ib qho zoo uas yuav tsum nrhiav kev pab kho mob es tsis txhob tso tseg lawv ua ib yam kab mob uas ib txwm muaj.

Vim li cas thiaj muaj tus mob VEXAS Syndrome? Dab tsi yog qhov ua rau muaj tus mob no?

Raws li peb tau tham txog ua ntej lawm, qhov ua rau qhov no yog kev hloov pauv caj ces hauv UBA1 gene . Kev hloov pauv caj ces yog kev hloov pauv hauv peb cov DNA. Cov kab ke DNA . Thaum cov cell faib, uas yog, thaum cov cell ua cov ntawv theej ntawm lawv tus kheej, qee zaum ib feem ntawm cov kab ke DNA no tuaj yeem nyob rau qhov chaw tsis raug, tsis tiav, lossis puas tsuaj. Thaum ntawd cov tsos mob ntawm cov mob caj ces tshwm sim.

Qhov uas tshwm sim rau ib tug neeg uas muaj VEXAS Syndrome yog tias UBA1 gene tsis ua haujlwm zoo, thiab E1 enzyme tsis tsim tawm raws li nws yuav tsum tau ua. Feem ntau, E1 enzyme zoo li "tus neeg ntxuav" hauv peb lub hlwb. Nws txoj haujlwm yog ntxuav cov khib nyiab, xws li cov protein qub thiab puas tsuaj, hauv cov hlwb. Tab sis thaum koj muaj VEXAS Syndrome, pab pawg "ntxuav" no tsis ua haujlwm zoo. Tom qab ntawd yuav ua li cas? Cov protein puas tsuaj thiab cov khib nyiab sib sau ua ke hauv cov hlwb. Thaum peb lub cev tiv thaiv kab mob pom cov khib nyiab no, nws xav tias muaj kev kis kab mob lossis kev hem thawj nyob ntawd. Tab sis vim tias tsis muaj kev kis kab mob nyob ntawd, lub cev tiv thaiv kab mob tawm tsam cov ntaub so ntswg noj qab haus huv. Qhov ntawd yog qhov ua rau mob. Xav txog nws zoo li lub tsheb thauj khib nyiab tsis ntxuav lub tsev thiab cov khib nyiab sib sau ua ke.

Leej twg muaj feem yuav mob VEXAS Syndrome ntau dua?

Cov kev tshawb fawb pom tau tias cov txiv neej muaj feem yuav mob no ntau dua. Nws kuj tseem pom ntau dua rau cov neeg hnub nyoog tshaj 50 xyoo. Qhov no txhais tau tias kev hloov pauv caj ces uas los nrog hnub nyoog yuav ua lub luag haujlwm.

Cov teeb meem uas yuav tshwm sim ntawm VEXAS Syndrome yog dab tsi?

Yog tias qhov mob VEXAS Syndrome ua rau koj cov pob txha tsis ua haujlwm , nws tuaj yeem ua rau koj lub cev tsis ua haujlwm zoo. Qhov no tuaj yeem ua rau koj tuag taus.

Nyob ntawm seb qhov mob o tuaj qhov twg, ib tus neeg uas muaj VEXAS Syndrome yuav muaj teeb meem kev noj qab haus huv ntxiv, xws li:

• Kab mob ntshav dawb (leukemia) ( ib hom mob qog ntshav)
• Myocarditis (mob ntawm cov leeg nqaij plawv)
• Kab mob ntshav tsis txaus
• Dermatitis ( mob ntawm daim tawv nqaij)
• Chondritis (mob ntawm cov pob txha mos)
• Vasculitis (mob ntawm cov hlab ntsha)
• Mob pob qij txha ( mob pob qij txha)
• Ntshav txhaws hauv cov hlab ntsha tob (Deep Vein Thrombosis - DVT)
• Colitis (mob ntawm txoj hnyuv loj)

Saib seb qhov no ua rau muaj mob hnyav npaum li cas. Yog vim li ntawd kev kuaj pom thiab kho mob thaum ntxov yog qhov tseem ceeb.

Cov kws kho mob kuaj mob VEXAS Syndrome li cas?

Tus kws kho mob yuav kuaj pom tus mob VEXAS Syndrome los ntawm kev kuaj koj lub cev thiab kuaj cov noob caj noob ces . Nws yuav tshuaj xyuas koj cov tsos mob kom zoo zoo thiab nug koj txog koj tau muaj cov tsos mob no ntev npaum li cas lawm.

Txawm li cas los xij, tib txoj kev kom paub tseeb tias koj puas muaj VEXAS Syndrome yog los ntawm kev kuaj noob caj noob ces. Hauv qhov no, koj tus kws kho mob yuav coj koj cov ntshav, tawv nqaij, plaub hau, lossis lwm yam nqaij thiab xa mus rau chav kuaj mob. Cov kws kho tshuab nyob ntawd yuav kuaj koj cov DNA kom pom tias koj puas muaj qhov kev hloov pauv hauv UBA1 gene uas ua rau muaj VEXAS Syndrome.

Cov kev kho mob rau VEXAS Syndrome yog dab tsi?

Cov kev kho mob tseem ceeb tam sim no rau tus mob no yog:

• Cov tshuaj Corticosteroids txo qhov o.
• Cov tshuaj tiv thaiv kab mob ua rau koj lub cev tiv thaiv kab mob qeeb.
• Yog tias koj cov pob txha tsis ua haujlwm zoo, tej zaum koj yuav xav tau kev hloov pob txha . Kev hloov pob txha kuj tseem tuaj yeem txo qhov hnyav ntawm qee yam mob autoimmune.

Koj kuj tseem yuav raug xa mus rau tus kws kho mob rheumatologist , tus kws kho mob uas tshwj xeeb hauv cov kab mob pob qij txha thiab cov kab mob autoimmune, uas tuaj yeem muab cov lus qhia tseeb tshaj plaws txog kev kho cov mob no.

Puas tiv thaiv tau VEXAS Syndrome?

Tu siab kawg li, tam sim no tsis muaj ib txoj kev twg los tiv thaiv tus kab mob no . Qhov kev hloov pauv hauv UBA1 gene tshwm sim tsis raws li qhov xav tau, yam tsis muaj laj thawj paub. Yog li peb tsis tuaj yeem tiv thaiv nws ua ntej.

Lub neej ntev npaum li cas ntawm ib tus neeg uas muaj VEXAS Syndrome?

Qhov no yog ib qho teeb meem rhiab heev. Qhov tseeb yog, txhua tus neeg txawv, thiab txoj kev uas VEXAS Syndrome cuam tshuam rau koj lub cev tuaj yeem txawv ntawm ib tus neeg mus rau ib tus neeg. Txawm li cas los xij, raws li peb tau hais ua ntej, qhov mob no tuaj yeem ua rau tuag taus yog tias tsis kho. Yog li tham nrog koj tus kws kho mob txog yam koj tuaj yeem cia siab thiab cov kev kho mob twg zoo tshaj plaws rau koj. Nws tuaj yeem pab koj tswj koj cov tsos mob thiab xa koj mus rau cov kws tshaj lij kev noj qab haus huv thiab lwm yam kev pabcuam txhawb nqa yog tias xav tau.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Yog tias koj muaj cov tsos mob ntawm VEXAS Syndrome, xws li kub taub hau, tawv nqaij tawm pob, lossis ua pa nyuaj, nco ntsoov mus ntsib kws kho mob. Vim tias VEXAS Syndrome ua rau muaj ntau yam tsos mob uas zoo li tsis muaj feem cuam tshuam, qee zaum nws yuav nyuaj rau kuaj mob thaum xub thawj. Txawm li cas los xij, mloog koj lub cev, thiab yog tias koj muaj cov tsos mob uas koj tsis nkag siab lossis tsis muaj feem cuam tshuam nrog koj lwm yam mob, tham nrog kws kho mob txog lawv.

Yog tias koj twb tau kuaj pom tias muaj VEXAS Syndrome lawm, thiab koj xav tias koj muaj cov tsos mob tshiab lossis koj cov tsos mob uas twb muaj lawm zuj zus tuaj, mus ntsib kws kho mob tam sim ntawd.

Xwm Ceev! Yog tias koj kub taub hau tshaj 103°F (39.5°C) ntev tshaj ob teev txawm tias koj tau kho mob hauv tsev lawm los xij, mus rau chav kho mob ceev. Yog tias koj ua tsis taus pa, mus rau tsev kho mob tam sim ntawd lossis hu rau 911 (lossis koj tus xov tooj xwm ceev hauv zos).

Kuv yuav tsum nug kuv tus kws kho mob li cas?

Thaum koj mus ntsib kws kho mob, npaj txhij nug cov lus nug zoo li no:

• Kuv puas muaj VEXAS Syndrome, los yog nws puas yog lwm yam mob?
• Kuv puas yuav tsum tau kuaj noob caj noob ces?
• Kuv xav tau kev kho mob hom twg?
• Kuv yuav tsum tau saib xyuas cov tsos mob lossis kev hloov pauv twg?
• Puas yog VEXAS Syndrome ua rau kuv lub neej muaj kev phom sij?

Cov lus nug no yuav yog qhov pib zoo rau koj pib tham nrog koj tus kws kho mob.

Thaum kawg, thaum kawg, nco ntsoov qhov no (Cov Lus Coj Mus Tsev)

VEXAS Syndrome yog ib yam kab mob autoimmune uas tsis tshua muaj tshwm sim los ntawm kev hloov pauv ntawm cov noob caj noob ces. Nws tuaj yeem ua rau mob thoob plaws koj lub cev thiab tuaj yeem ua rau tuag taus yog tias tsis kho. Tab sis tsis txhob tso tseg kev cia siab . Koj tus kws kho mob tuaj yeem pab koj nrhiav kev kho mob kom zoo los tswj koj cov tsos mob.

Yog koj muaj tej tsos mob ntawm VEXAS Syndrome, mus ntsib kws kho mob. Ntseeg koj tus kheej, mloog koj lub cev. Tsis txhob tsis quav ntsej cov tsos mob xws li kub taub hau, pob khaus, thiab ua pa nyuaj. Yog kuaj pom thaum ntxov thiab kho kom zoo, koj tuaj yeem ua neej noj qab nyob zoo dua.

VEXAS Syndrome, Cov Kab Mob Autoimmune, O, Kev Hloov Pauv Gene, UBA1 Gene, Pob Txha