Koj tus menyuam puas feem ntau kub taub hau yam tsis muaj laj thawj? Cia peb kawm txog cov kab mob SAIDs (Systemic Autoinflammatory Diseases) no!

Puas yog koj tus me nyuam me me qee zaum tsuas yog kub taub hau xwb yam tsis muaj mob khaub thuas lossis lwm yam mob? Puas yog qhov kub taub hau tuaj, ploj mus hauv ob peb hnub, thiab rov tshwm sim dua ob peb hnub tom qab? Ib txhia niam txiv ntsib cov kev paub zoo li no. Yog li hnub no peb yuav tham txog qhov ua rau kub taub hau ntau zaus thiab nyuaj nrhiav. Qhov no hu ua SAIDs (Systemic Autoinflammatory Diseases) . Yav dhau los, qhov no hu ua '(Periodic Fever Syndromes)' lossis '(Recurrent Fever Syndromes)'.

Cov SAIDs no yog dab tsi? Cia peb nkag siab yooj yim!

Hais yooj yim xwb, SAIDs yog ib pawg kab mob uas tshwm sim los ntawm kev ua haujlwm tsis zoo lossis teeb meem kev tswj hwm hauv peb lub cev lub cev tiv thaiv kab mob . Hauv qhov no, yog tsis muaj kev kis kab mob sab nraud (xws li tus kab mob lossis cov kab mob me me), peb lub cev tsuas yog tsim kev o. Zoo li peb lub cev tiv thaiv kab mob tab tom ua haujlwm ntau dhau.

Xav txog nws, peb muaj ib pawg tub rog hauv peb lub cev (qhov ntawd yog lub cev tiv thaiv kab mob). Lawv txoj haujlwm yog tawm tsam cov kab mob ua rau muaj kab mob thaum lawv nkag los. Tab sis hauv lub cev ntawm ib tus neeg uas muaj SAIDs, cov tub rog no qee zaum tsuas yog ntxhov siab thiab ua rau muaj teeb meem hauv lub cev.

Tam sim no tej zaum koj yuav xav paub seb qhov no puas yog ib yam dab tsi zoo li '(cov kab mob autoimmune)' (uas yog, cov kab mob autoimmune). Piv txwv li, '(rheumatoid arthritis)' lossis '(lupus)' zoo sib xws. Tsis yog, ob qho no txawv me ntsis. Hauv cov kab mob autoimmune, qhov tshwm sim yog tias peb lub cev tiv thaiv kab mob tau txais lossis hloov kho tau ua yuam kev tawm tsam nws cov hlwb noj qab haus huv. Tab sis hauv SAIDs, qhov teeb meem nyob nrog lub cev tiv thaiv kab mob uas yug los.

Cov kab mob SAIDs tsis tshua muaj ntau dua li cov kab mob autoimmune. Feem ntau ntawm lawv yog los ntawm noob caj noob ces , txhais tau tias lawv yog los ntawm kev hloov pauv caj ces lossis variant.

Feem ntau cov kab mob SAIDs pib thaum lawv tseem yog menyuam yaus lossis thaum lawv tseem yog menyuam yaus . Tiamsis qhov ntawd tsis yog li ntawd tas li. Tej zaum koj tus menyuam yuav muaj mob kub taub hau lossis mob hnyav thiab lwm yam tsos mob. Tej zaum koj tus menyuam yuav tsis muaj tsos mob dab tsi li ntawm cov mob uas pib mob. Tsis muaj tshuaj kho tau cov kab mob SAIDs. Txawm li cas los xij, muaj cov kev kho mob uas tuaj yeem pab tswj cov tsos mob hauv feem ntau.

Cov hom SAIDs tseem ceeb yog dab tsi?

Cov kws tshawb nrhiav tau txheeb xyuas txog li 60 hom kab mob SAIDs, thiab tseem muaj ntau hom kab mob uas tab tom raug tshawb pom. Cia peb saib qee hom kab mob uas feem ntau pom.

• Kab mob ua npaws Mediterranean hauv tsev neeg (FMF): Qhov no yog hom kab mob ua npaws uas rov tshwm sim ntau tshaj plaws los ntawm noob caj noob ces. Nws tuaj yeem ua rau mob o ntawm lub plab, hauv siab, thiab pob qij txha ntawm tus menyuam .
• Mob ua npaws tas li, mob aphthous-stomatitis, mob pharyngitis, mob adenitis (PFAPA): Feem ntau qhov no pib rau cov menyuam yaus ua ntej hnub nyoog 4 xyoos. Tus mob no yuav ploj mus yam tsis muaj kev cuam tshuam tom qab hnub nyoog 10 xyoos.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Qhov no tuaj yeem tshwm sim thaum yau, thaum hluas, thiab qee zaum txawm tias laus.
• Mevalonate kinase deficiency (MKD): Yav tas los hu ua hyper-IgD syndrome, qhov no feem ntau pib rau cov menyuam mos hnub nyoog qis dua 1 xyoos.
• Cov kab mob autoinflammatory uas cuam tshuam nrog NLRP3: Qhov no yav dhau los hu ua cryopyrin-associated periodic syndromes (CAPS). Muaj peb hom kab mob me me hauv qhov no.
• Kab mob Still uas pib rau cov neeg laus (AOSD): Qhov no yog ib hom kab mob systemic juvenile idiopathic arthritis (JIA) uas pib rau cov neeg laus.
• Kab mob granulomatous cuam tshuam nrog NOD-2 (Blau syndrome): Qhov no feem ntau pib ua ntej hnub nyoog 4 xyoos. Nws feem ntau cuam tshuam rau daim tawv nqaij, qhov muag, thiab pob qij txha ntawm tus menyuam.

Cov tsos mob ntawm SAIDs yog dab tsi? Peb yuav paub lawv li cas ?

Cov tsos mob ntawm tus kab mob SAID feem ntau pib thaum yau. Cov tsos mob feem ntau thiab pom tseeb tshaj plaws yog kub taub hau tas li lossis kub taub hau ib ntus . Feem ntau cov neeg tsis muaj tsos mob ntawm kev mob. Txawm li cas los xij, tej zaum yuav muaj lwm cov tsos mob tshwj xeeb rau txhua hom kab mob SAID.

Cia peb saib cov tsos mob tshwj xeeb ntawm ntau hom SAID uas tau tham saum toj no:

• FMF: Qhov no tuaj yeem ua rau mob hnyav hauv plab, hauv siab, thiab pob qij txha ntawm tus menyuam, nrog rau o . Qee zaum, pob khaus tuaj yeem tshwm sim ntawm ob txhais ceg qis lossis pob luj taws. Xav txog, ib tug menyuam tam sim ntawd mob plab thiab kub taub hau. Thaum cov niam txiv txhawj xeeb thiab coj lawv mus rau tsev kho mob, lawv yuav xav tias nws yog mob appendicitis. Tab sis tom qab ntawd nws ploj mus thiab rov qab los tom qab ob peb hnub.
• PFAPA: Qhov no tuaj yeem ua rau muaj cov tsos mob xws li mob caj pas, mob qhov ncauj, thiab o ntawm cov qog ntshav hauv caj dab .
• KEV NTSUAM: Qhov no tuaj yeem ua rau mob khaub thuas, mob nqaij hauv siab thiab caj npab , thiab ua pob liab liab uas pib ntawm caj npab thiab ceg thiab kis mus thoob plaws lub cev.
• MKD: Hauv qhov no thiabTej zaum koj yuav tsis xis nyob nrog rau mob khaub thuas, mob taub hau, mob plab, tsis xav noj mov, thiab cov tsos mob zoo li mob khaub thuas.
• Cov kab mob NLRP3: Cov no tuaj yeem ua rau muaj cov tsos mob xws li ua pob khaus ntawm daim tawv nqaij, mob taub hau, mob nqaij, mob pob qij txha, thiab qhov muag liab (conjunctivitis) .
• AOSD: Qhov no yog ib qho mob uas feem ntau ua rau tawv nqaij khaus, mob pob qij txha, thiab mob nqaij . Ib txhia neeg kuj tseem yuav mob caj pas, mob plab, qaug zog heev, thiab mob lub cev.
• Kab mob Blau: Qhov no tuaj yeem ua rau mob tawv nqaij ntawm koj tus menyuam txhais tes, ceg, lossis lub cev . Nws kuj tseem tuaj yeem ua rau mob pob qij txha thiab mob qhov muag .

Vim li cas cov SAIDs no thiaj tshwm sim? Dab tsi yog cov ua rau muaj?

Feem ntau cov kab mob SAIDs yog cov kab mob caj ces . Uas yog, txhua yam kab mob yog tshwm sim los ntawm kev hloov pauv (variant) hauv ib qho gene tshwj xeeb.

Cia peb saib cov noob caj noob ces uas cuam tshuam rau ntau hom SAID loj:

• FMF: Qhov no yog tshwm sim los ntawm ib tug gene hu ua `(MEFV gene)`. Cov gene no qhia txog yuav ua li cas los tsim ib tug protein hu ua `(pyrin)`.
• PFAPA: Qhov laj thawj tseeb rau qhov no tseem tsis tau pom .
• TRAPS: Qhov no yog tshwm sim los ntawm ib tug gene hu ua '(TNFRSF1A gene)'. Qhov no yog qhov muab cov lus qhia rau kev ua ib tug protein hu ua '(tumor necrosis factor receptor - TNFR)'.
• MKD: Qhov laj thawj rau qhov no yog ib qho gene hu ua '(MVK gene)'. Qhov no qhia kom muaj cov protein hu ua '(mevalonic kinase)'.
• Cov kab mob NLRP3: Qhov no yog tshwm sim los ntawm ib tug gene hu ua '(NLRP3 gene)'. Qhov no muab cov lus qhia los ua ib tug protein hu ua '(cryopyrin)'.
• AOSD: Qhov laj thawj tseeb rau qhov no tseem tsis tau pom .
• Kab mob Blau: Qhov no yog tshwm sim los ntawm ib hom noob hu ua '(NOD2 gene)'. Qhov no qhia kom muaj ib hom protein hu ua '(NOD2)'.

Cov SAIDs no puas ua rau muaj lwm yam teeb meem?

Yog, qhov no tuaj yeem ua rau muaj teeb meem yog tias tsis kho kom zoo . Yog tias kev o ntawm lub cev tseem txuas ntxiv mus, nws tuaj yeem ua rau muaj tus mob hu ua "amyloidosis." Uas yog, ib hom protein tau tso rau hauv peb lub raum. Qhov no tuaj yeem ua rau lub raum puas tsuaj mus tas li . Yog vim li ntawd nws thiaj li tseem ceeb rau kev kuaj mob thaum ntxov thiab tau txais kev kho mob.

Tus kws kho mob yuav kuaj mob SAIDs no li cas?

Qhov tseeb, kev kuaj mob cov kab mob autoinflammatory no yuav nyuaj me ntsis , vim tias lawv cov tsos mob tuaj yeem ua raws li lwm yam mob hnyav xws li lupus lossis mob qog noj ntshav xws li lymphoma. Yog li ntawd, nws yog ib qho tseem ceeb kom mus ntsib kws kho mob uas muaj kev cob qhia tshwj xeeb hauv cov kab mob inflammatory, tus kws kho mob rheumatologist, kom kuaj mob thiab tswj hwm koj tus menyuam tus mob kom zoo.

Koj tus menyuam tus kws kho mob pob qij txha yuav siv ntau yam los kuaj mob SAIDs. Nws yuav nug koj txog koj tus menyuam cov tsos mob thiab seb puas muaj leej twg hauv koj tsev neeg muaj cov mob no (keeb kwm tsev neeg). Tus kws kho mob yuav xav tias koj tus menyuam yuav muaj tus mob ua npaws tas li yog tias:

• Yog tias tus menyuam muaj mob kub taub hau ntau zaus .
• Yog tias leej twg hauv tsev neeg muaj keeb kwm ntawm kev kub taub hau ntau zaus zoo li no .
• Yog tias tus menyuam koom nrog ib pawg neeg uas muaj feem yuav kub taub hau tas li zoo li no .

Yuav ua li cas thiaj kuaj tau qhov no?

Yuav kom paub tseeb tias koj tus menyuam mob pob qij txha, tus kws kho mob pob qij txha yuav pom zoo kom kuaj ntau yam. Cov no suav nrog:

• Kev kuaj mob hauv chav kuaj: Cov kev kuaj xws li "C-reactive protein (CRP)" thiab "complete blood count (CBC)" siv tau los kuaj seb puas muaj mob o hauv lub cev. Cov kev kuaj no pom tias muaj mob thaum lub cev kub taub hau "nres" thiab rov qab mus rau qhov qub thaum lub cev kub taub hau tas lawm.
• Kuaj zis: Kev kuaj zis protein tuaj yeem ua tau los xyuas seb puas muaj protein ntau dhau hauv cov zis. Hauv MKD, kev kuaj zis uas saib cov organic acids (cov kua qaub uas muaj ib lub carbon atom) tuaj yeem qhia tau tias muaj cov mevalonic acid ntau dhau.
• Kev kuaj noob caj noob ces: Qhov no tuaj yeem kuaj xyuas seb puas muaj cov noob caj noob ces sib txawv. Txawm li cas los xij, qee tus menyuam uas muaj SAIDs yuav tsis muaj cov noob caj noob ces sib txawv uas pom, uas tuaj yeem ua rau cov txiaj ntsig kuaj tsis zoo.

Cov kev kho mob rau cov SAIDs no yog dab tsi?

Kev kho mob rau SAIDs sib txawv nyob ntawm hom thiab qhov hnyav ntawm tus mob . Tsis muaj kev kho rau cov mob no. Txawm li cas los xij , koj tus menyuam cov tsos mob feem ntau tuaj yeem tswj tau nrog tshuaj . Yog tias koj tus menyuam tsuas muaj ob peb zaug mob hauv ib xyoos, koj yuav muab tau cov tshuaj tua kab mob rau lawv xws li cov tshuaj nonsteroidal anti-inflammatory drugs (NSAIDs) los pab txo lawv cov tsos mob. Txawm li cas los xij, yog tias tus mob hnyav dua, koj tus kws kho mob yuav qhia lwm txoj kev kho mob.

Nov yog qee yam ntawm lawv:

• FMF: Siv tau ib yam tshuaj hu ua '(colchicine)' los tswj qhov no. Qhov no yuav txo qhov o. Yog tias tus me nyuam noj tsis tau colchicine, sim siv ib hom tshuaj '(biologic)' hu ua '(canakinumab)'.
• PFAPA: Cov tshuaj steroids (feem ntau yog prednisone) tuaj yeem muab tau los ua kom lub sijhawm luv dua ntawm PFAPA "kev tawm tsam." Rau qee tus menyuam yaus, cimetidine, ib yam tshuaj siv los kho cov mob plab, kuj tseem tuaj yeem pab tswj cov tsos mob.
• KEV TXHEEB XYUAS: Ib hom tshuaj hu ua '(canakinumab)' feem ntau siv tau zoo rau qhov no. Tsis tas li ntawd, cov tsos mob tuaj yeem txo tau los ntawm kev siv cov tshuaj tiv thaiv kev o uas tus kws kho mob tau sau tseg, xws li '(glucocorticoids)'.
• MKD:Canakinumab kuj yog ib txoj kev kho mob zoo rau MKD. Kev muab cov NSAIDs lossis steroids thaum lub sijhawm "tawm tsam" kuj tseem yuav pab tau.
• Cov kab mob NLRP3: Cov tshuaj immunomodulators xws li canakinumab, rilonacept, thiab anakinra raug siv zoo rau qhov no.
• AOSD: Muaj ntau hom tshuaj tiv thaiv kev o uas siv rau AOSD. Piv txwv li, muaj cov tshuaj steroids, cov tshuaj kho mob rheumatic uas hloov kho kab mob (DMARDs), thiab cov tshuaj biologics.
• Blau syndrome: Nyob ntawm seb koj tus menyuam cov tsos mob li cas, lawv yuav sim siv cov tshuaj immunosuppressants, tumor necrosis factor (TNF) inhibitors, thiab/lossis cov tshuaj pleev qhov muag.

Cov mob SAIDs no puas yuav nyob mus ib txhis? Los yog lawv puas yuav ploj mus?

Qee cov mob autoinflammatory yog cov mob uas yuav kav ntev mus ib txhis . Lwm cov mob yuav kav ntev li ob peb xyoos thiab ploj mus thaum laus. Qee cov mob yuav kav ntev mus ib txhis, tab sis dhau sijhawm, qhov zaus thiab qhov hnyav ntawm cov mob yuav txo qis. Koj tus menyuam tus kws kho mob tuaj yeem muab cov ntaub ntawv tshwj xeeb rau koj txog koj tus menyuam tus mob.

Kev tsa ib tug me nyuam uas muaj tus kab mob autoinflammatory tuaj yeem nyuaj heev. Yog tias koj muaj SAID, koj tuaj yeem siv koj tus kheej cov kev paub dhau los los txhawb kev saib xyuas koj tus menyuam zoo dua. Koj tuaj yeem siv koj cov kev paub dhau los los pab koj tus menyuam hloov kho rau qhov mob no mus tas li.

Qhov tseem ceeb tshaj plaws yog nrhiav kev kho mob los ntawm cov kws tshwj xeeb uas muaj kev paub txog SAIDs. Cov kws kho mob zoo li no tuaj yeem pab tswj koj tus menyuam cov tsos mob thiab pab lawv muaj lub neej thaum yau zoo tshaj plaws.

Cov Lus Xaus Kawg coj mus tsev

Yog li, kuv vam tias koj muaj qee lub tswv yim txog SAIDs uas peb tau tham txog hnub no. Nco ntsoov, yog tias koj tus menyuam muaj kub taub hau ntau zaus, tsis paub vim li cas, nrog kws kho mob tham txog nws . Tsis txhob tsuas yog tsis quav ntsej nws, xav tias nws yog ib txwm muaj.

• SAIDs yog ib pawg kab mob uas feem ntau ua rau kub taub hau thiab yog los ntawm teeb meem nrog lub cev tiv thaiv kab mob.
• Cov no feem ntau yog los ntawm noob caj noob ces thiab pib thaum tseem hluas.
• Txawm hais tias tsis muaj kev kho mob mus tas li rau qhov no, muaj kev kho mob uas tuaj yeem tswj cov tsos mob.
• Nws yog ib qho tseem ceeb uas yuav tsum nrhiav kev kuaj mob thiab kev kho mob kom zoo los ntawm tus kws kho mob tshwj xeeb (Rheumatologist).
• Yog tias pib kho mob thaum ntxov, cov teeb meem xws li amyloidosis tuaj yeem tiv thaiv tau thiab tus menyuam tuaj yeem ua neej nyob li qub.

Yog koj muaj lus nug ntxiv txog qhov no, tsis txhob yig nug koj tus kws kho mob hauv tsev neeg lossis kws kho menyuam yaus.

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