Ðe asrã dzea viwòa dzi enuenua? Ðe wòate ŋu atso SAIDs (Systemic Autoinflammatory Diseases) gbɔa?

Oo, ɖe viwò suea xɔa asrã enuenu le ŋkeke siawo mea? Ne èsusu be yeƒe lãme sẽ ko la, ɖe wògafɔna akea? Ɣeaɖewoɣi la, ɖewohĩ menye dɔlékui alo dɔlékui aɖe si bɔ gbɔe esia tsona o. Egbea míele nu ƒom tso nɔnɔme aɖe si sesẽ vie ŋu, gake ele vevie ŋutɔ be míanya nu tso eŋu. Woyɔa esia be SAIDs, si gɔmee nye Systemic Autoinflammatory Diseases . Tsã la, woyɔa esia be `(Periodic Fever Syndromes)` alo `(Recurrent Fever Syndromes)`.

Nukae nye SAIDs? Mina míase egɔme bɔbɔe.

Kpuie ko la, SAID nye dɔlélewo ƒe hatsotso aɖe si tso míaƒe ŋutilã ƒe dɔlélenutsiŋutete si wodzina ƒe dɔmawɔmawɔ nyuie alo ɖoɖowɔwɔ ƒe kuxi aɖe gbɔ . Esia tae ɖevi ate ŋu axɔ asrã enuenu ne dɔlékui aɖeke mele eŋu o (abe dɔlékui alo dɔlékui ene).

Nu vevitɔe nye be mele be woatɔtɔ nɔnɔme siawo siwo woyɔna be SAIDs kple `(Autoimmune Diseases)` (le kpɔɖeŋu me `(Rheumatoid Arthritis)` alo `(Lupus)` si ŋu ɖewohĩ èse nu tsoe. Le `(Autoimmune)` dɔlélewo me la, le vodada aɖewo le míaƒe ŋutilã ƒe `(adaptive immune system)` me ta la, ɖoɖo ma dzea eya ŋutɔ ƒe lãmenugbagbevi siwo le lãmesẽ me dzi. Gake le SAID-wo me la, nu bubu aɖe dzɔna.

SAID-tikewo mebɔ kura o wu dɔléle siwo dɔlélenutsiŋutete hena vɛ. Zi geɖe la domenyiŋusẽfianu mee wotsona eye domenyiŋusẽfianu ƒe tɔtrɔ gbɔe wotsona .

Zi geɖe la, gake menye ɣesiaɣie o, SAID ƒe dzesiwo dzea egɔme ne ɖevi nye ɖevi sue ŋutɔ, esime wònye vidzĩ alo ɖevi si le zɔzɔm . Viwò ateŋu akpɔ "ɣeyiɣiwo" ƒe dzoxɔxɔ kple dzesi bubuwo kple nɔnɔme sia. Le ɣeyiɣi siawo dome la, ɖewohĩ dzesi aɖeke manɔ viwòa ŋu kura o. Togbɔ be atike aɖeke meli na SAID-dɔlékuiwo o hã la , zi geɖe la, atikewɔmɔnu aɖewo li siwo akpe ɖe viwòa ƒe dzesiwo nu.

Nukawoe nye SAID ƒomevi veviwo?

Numekulawo de dzesi SAID ƒomevi siwo ade 60, eye wogale yeyewo dim kokoko. SAID ƒomevi siwo bɔ wu dometɔ aɖewoe nye esi:

• Ƒomea ƒe Mediterranea-ƒua ƒe dzoxɔxɔ (FMF): Esiae nye dɔléle si bɔ wu le domenyiŋusẽfianu ƒe dɔléle siwo gbugbɔna dzɔna la dome. Ate ŋu ana ɖeviwo ƒe ƒodo, akɔta, kple ƒunukpeƒewo navuvu vevesesetɔe.
• Dzoxɔxɔ si nɔa ame ŋu ɣeaɖewoɣi, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Zi geɖe la, esia dzea egɔme tso ɖevi suewo dzi hafi woxɔa ƒe 4. Ɣeaɖewoɣi la, nɔnɔmea ate ŋu anyo ɖe edzi le eɖokui si le ƒe 10 xɔxɔ vɔ megbe.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Esia ate ŋu adzɔ le ɖevime, ƒewuiwo, alo ame tsitsi gɔ̃ hã me.
• Mevalonate kinase ƒe anyimanɔmanɔ (MKD): Tsã la, woyɔnae be ``Hyper-IgD syndrome'', zi geɖe la, esia dzea egɔme hafi ɖevia xɔ ƒe ɖeka.
• Dɔléle siwo do ƒome kple NLRP3:Woyɔa esia tsã be `(Cryopyrin-associated periodic syndromes - CAPS)`. Ƒomevi sue etɔ̃ bubuwo hã le esia me.
• Still ƒe dɔléle si dzea egɔme tso ame tsitsiwo gbɔ (AOSD): Abe alesi ŋkɔa ɖee fia ene la, esia nye ɖevimenɔɣi ƒe nɔnɔme si dzea egɔme tso ame tsitsiwo gbɔ ``Systemic Juvenile Idiopathic Arthritis (JIA)``.
• NOD-2-associated granulomatous disease (Blau syndrome): Zi geɖe la, esia dzea egɔme hafi wòxɔ ƒe 4. Edzea ɖevia ƒe ŋutigbalẽ, ŋku, kple ƒunukpeƒewo koŋ.

Nukae nye SAID-dɔlékuiwo ƒe dzesiwo?

Zi geɖe la, SAID ƒe dzesiwo dzea egɔme tso ɖevime. Dzesi vevitɔ kple esi bɔ wue nye dzoxɔxɔ si nɔa ame ŋu ɣeaɖewoɣi . Le asrãɣiwo la, zi geɖe la, amewo ate ŋu anɔ lãmesẽ me eye dzesi aɖeke menɔa wo ŋu o. Gake dzesi bubuwo ate ŋu anɔ anyi siwo ku ɖe SAID ƒomevi ɖesiaɖe koŋ ŋu. Na míakpɔ nusiwo wonye ɖa:

• FMF: Esia ate ŋu ana ɖevia ƒe ƒodo, akɔta, kple ƒunukpeƒewo navuvu eye wòase veve vevie . Ate ŋu adzɔ hã be ʋuʋudedi nanɔ afɔ siwo le ete alo afɔkpodziwo ŋu.
• PFAPA: Esia ate ŋu ana ŋɔti nave ame, nu me naxɔ abi, eye ʋuka siwo le kɔ me navuvu . Le kpɔɖeŋu me, ne viwòa le dzesiwo abe aɖuɖɔtoedɔléle enuenu kple nu me abi ene ɖem fia la, ate ŋu anye PFAPA.
• MƆXƆWO: Esia ate ŋu ana lãmekawo nase veve eye lãmekawo nase veve le akɔta/akɔta kple abɔwo me . Ate ŋu ana ʋuʋudedi dzĩ si le veve sem hã nadze egɔme tso abɔwo kple afɔwo dzi eye wòakaka ɖe ŋutilãa me.
• MKD: Esia ate ŋu ahe dzesiwo abe vuvɔ, taɖuame, dɔgbo ƒe veve, nuɖuɖu ƒe dzodzro, kple dzesi siwo ɖi dzamezã ene vɛ .
• NLRP3 dɔlélewo: Nɔnɔme sia ate ŋu ahe ŋutigbalẽ ƒe ʋuʋu, taɖuame, lãmetutudɔ, ƒunukpeƒeveame, kple ŋku/ŋku ƒe ʋuʋu si le pink vɛ .
• AOSD: Esia nye dɔléle si bɔ si hea ŋutigbalẽ ƒe ʋuʋu, ƒunukpeƒeveame, kple lãmekawo ƒe vevesese vɛ . Ame aɖewo hã ate ŋu ase veve le ŋɔti me, dɔgbo nave wo, ɖeɖi nate wo ŋu, eye woƒe lãme nasẽ.
• Blau ƒe dɔléle: Esia ate ŋu ana ŋutigbalẽ naxɔ viwòa ƒe abɔwo, afɔwo, alo titina . Ate ŋu ana ƒunukpeƒewo nase veve eye ŋku nase veve hã .

Nukawoe hea SAID-dɔlékuiwo vɛ?

SAID geɖe nye domenyiŋusẽfianu ƒe dɔlélewo . Eyae nye be domenyiŋusẽfianu aɖe koŋ ƒe tɔtrɔ (to vovo) aɖe koŋ hea dɔléle ɖesiaɖe vɛ. Tsɔe be mɔfiame (domenyiŋusẽfianu) aɖewo le míaƒe ŋutilã si siwo gblɔa alesi míawɔ dɔ ɖesiaɖe na mí, eyata ne ŋɔŋlɔdzesi ɖeka si le agbalẽ ma me mesɔ o la, ate ŋu ana dɔ susɔea nato vovo.

SAID ƒomevi veviwo kple domenyiŋusẽfianu siwo do ƒome kpli woe nye esi:

• FMF:Domenyiŋusẽfianu `MEFV` ye naa mɔfiamewo le alesi woawɔ protein `(pyrin)` ŋu.
• PFAPA: Womekpɔ nya nusi tututu gbɔ esia tso haɖe o .
• TRAPS: Domenyiŋusẽfianu si nye `TNFRSF1A`. Esia fiaa mɔ protein si woyɔna be `(tumor necrosis factor receptor - TNFR)` ƒe wɔwɔ.
• MDK: Domenyiŋusẽfianu si woyɔna be `MVK`. Esia fiaa mɔ protein si woyɔna be `(mevalonic kinase)` ƒe wɔwɔ.
• NLRP3 dɔlélewo: Domenyiŋusẽfianu si nye `NLRP3`. Esia fiaa mɔ protein si woyɔna be `(cryopyrin)` ƒe wɔwɔ.
• AOSD: Womeke ɖe nusi tututu gbɔ esia tso ŋu haɖe o .
• Blau ƒe dɔléle: Domenyiŋusẽfianu si nye `NOD2`. Esia fiaa mɔ protein si woyɔna be `(NOD2)` ƒe wɔwɔ.

Ele vevie be míase egɔme be ne domenyiŋusẽfianu gbɔe nusiawo tso la, menye dzilawo ƒe vodadae o. Esiawo nye nugbagbewo ƒe dɔwɔwɔ siwo sesẽ ŋutɔ.

Kuxi siwo SAID-dɔlékuiwo hena vɛ

Ele vevie ŋutɔ be woakpɔ atike nyuitɔ na nɔnɔme siawo. Elabena, ne dɔdzẽ yi edzi le ŋutilã me la, ate ŋu ahe nɔnɔme aɖe si woyɔna be `(Amyloidosis) vɛ. `(Amyloidosis)` nye protein si le ayiku me . Esia ate ŋu agblẽ nu le ayikuawo ŋu tegbee . Eya ta ne dzesiwo le eŋu la, ele vevie be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo enumake.

Aleke wowɔna dea dzesi SAID-wo?

Ne míagblɔe tututu la, SAID-dɔlékuiwo didi ate ŋu asesẽ vie elabena dzesiawo ate ŋu asɔ kple dɔléle sesẽ bubuwo abe lupus alo ʋumenugbagbeviɣidɔ ene. Eyata ele vevie be nàkpɔ ɖɔkta si xɔ hehe tɔxɛ le dɔdzẽ ƒe dɔlélewo ŋu (lãmekaŋutinunyala) be wòakpɔ viwòa ƒe nɔnɔmea nyuie ahakpɔ edzi.

Viwò ƒe lãmekaŋutinunyala azã nu geɖe atsɔ anya SAID-dɔlékuiwo. Abia nya tso viwòa ƒe dzesiwo ŋu kple nenye be nɔnɔme siawo nɔ ame aɖe ŋu le wò ƒomea me (ƒome ƒe ŋutinya si wodzi le dzɔdzɔme nu). Ðɔktaa ate ŋu asusui be Periodic Fever Syndrome le viwòa ŋu ne:

• Ne asrã le ŋuwò enuenu
• Ne ƒomea me tɔ aɖe xɔ asrã ɣeaɖewoɣi abe esia ene kpɔ
• Ne èle amewo ƒe ƒuƒoƒo aɖe si me asrã mawo te ŋu dzena bɔbɔe la me

Dodokpɔ kawoe wozãna?

Wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàwɔ dodokpɔ geɖe atsɔ aɖo kpe dɔlélea dzi. Esiawo ate ŋu anye:

• Dodokpɔwo le dodokpɔxɔ me: Dodokpɔwo abe `(C-reactive protein - CRP)` kple `(Complete Blood Count - CBC)` ateŋu akpɔe ɖa be dɔdzẽ le ŋutilã me hã. Dodokpɔ siawo nye `(positive)` ne asrã le ame ŋu, eye wogatrɔna yia nɔnɔme si sɔ me ne asrãa nu yi.
• Aɖuɖɔtoe dodokpɔ:Woate ŋu awɔ protein dodokpɔ le aɖuɖɔ me atsɔ akpɔe ɖa be protein sɔ gbɔ ɖe aɖuɖɔ me hã. Le MKD me la, aɖuɖɔ me dodokpɔ hena organic acids aɖee afia be mevalonic acid ƒe agbɔsɔsɔ dzi ɖe edzi.
• Domenyiŋusẽfianu dodokpɔ: Esia ate ŋu alé ŋku ɖe domenyiŋusẽfianu ƒe vovototowo ŋu. Gake ɖewohĩ domenyiŋusẽfianu ƒe tɔtrɔ si ŋu woke ɖo la manɔ ɖevi aɖewo siwo ŋu SAID le ŋu o. Eyata ne dodokpɔa me tsonu nye ``negative`` hã la, womate ŋu agbe SAID ƒe mɔnukpɔkpɔ o.

Aleke wowɔa nu ɖe ​​SAID-dɔlékuiwo ŋui?

Atikewɔwɔ na SAIDs toa vovo le dɔlélea ƒomevi kple eƒe sesẽ nu . Togbɔ be womate ŋu ada dɔléle siawo keŋkeŋ o hã la, woate ŋu aɖu ɖevia ƒe dzesiwo dzi nyuie ne wozã atikewo . Le kpɔɖeŋu me, ne zi ʋɛ aɖewo koe viwòa xɔa dzamezã siawo le ƒea me la, zi geɖe la, vevesesenutsitikewo kple atike siwo me steroid mele o (NSAIDs) ate ŋu aɖu dzesiawo dzi. Gake ne dɔlélea nu sẽ wu la, ɖɔkta ate ŋu agblɔ atike bubu siwo woate ŋu azã.

Atikewɔwɔ mawo dometɔ aɖewoe nye esi:

• FMF: Woateŋu atsɔ atike si woyɔna be `(Colchicine)` ada esia be wòaɖe abi dzi akpɔtɔ. Ne màte ŋu ano `(Colchicine)` o la, àte ŋu ate atike ƒomevi aɖe si woyɔna be `(Canakinumab)` kpɔ.
• PFAPA: Woate ŋu aɖe PFAPA ƒe dzoxɔxɔ dzi akpɔtɔ ne wona steroid (zi geɖe la, prednisone). Ðevi aɖewo te ŋu ɖu woƒe dzesiwo dzi to cimetidine, si nye atike si wozãna tsɔ daa dɔgbo ƒe abiwo zazã me.
• TRAPS: Atike si nye `(Canakinumab)` wɔa dɔ ŋutɔ na TRAPS. Azɔ hã, atike siwo woŋlɔ na ame siwo tsia dɔdzẽ nu abe `(Glucocorticoids)` hã ​​ate ŋu akpe ɖe ame ŋu be woaɖe dzesiawo dzi akpɔtɔ.
• MKD: `(Canakinumab)` nye tiatia nyui aɖe na MKD dada. `(NSAIDs)` alo `(Steroids)` zazã le asrãɣi hã ateŋu akpeɖeŋu.
• NLRP3 dɔlélewo: Wozãa ``Dɔlélenutsiŋutete'' atike bubuwo abe ``Canakinumab'' kple ``Rilonacept'' kple ``Anakinra'' dzidzedzetɔe tsɔ daa NLRP3 dɔlélewo.
• AOSD: Wozãa atike vovovo siwo tsia dzodzodzoetsitsi nu na AOSD. Esiawo dometɔ aɖewoe nye `(Steroids)`, `(Dɔléle-trɔtrɔ Atike Siwo Tsi Tre Ðe Lãmetsiŋusẽ Ŋu - DMARDs)`, kple `(Biologics)`.
• Blau ƒe dɔléle: Le ɖevia ƒe dzesiwo nu la, woate ŋu ate dɔlélenutsiŋutete ƒe dɔlékuiwutikewo, tumor necrosis factor (TNF) inhibitors, kple/alo ŋkutike siwo wozãna ɖe ŋku dzi la kpɔ.

Ðe SAIDs ƒe nɔnɔmea nyona ɖe edzi le eɖokui sia?

SAID aɖewo ate ŋu anɔ anyi le woƒe agbemeŋkekewo katã me . Bubuwo ate ŋu anɔ anyi ƒe ʋɛ aɖewo eye woƒe lãme asẽ ne wole tsitsim. Togbɔ be nɔnɔme aɖewo nɔa ame ƒe agbemeŋkekewo katã me hã la, dzoxɔxɔ ƒe dzɔdzɔ zi geɖe ate ŋu aɖe akpɔtɔ le ɣeyiɣi aɖe megbe eye dzesiawo magasẽ ɖe edzi o . Wò ɖɔkta ate ŋu agblɔ nya aɖewo koŋ tso viwòa ƒe nɔnɔme tɔxɛa ŋu.

Ðevi si ŋu SAID le dzi kpɔkpɔ ate ŋu asesẽ. Ðewohĩ wò ŋutɔe GBLƆ, gake àte ŋu azã wò ŋutɔ wò nuteƒekpɔkpɔwo atsɔ akpe ɖe viwòa dzi kpɔkpɔ ŋu eye nàkpe ɖe eŋu wòanɔ te ɖe nɔnɔme sia si le eƒe agbemeŋkekewo katã me nu.

Nu vevitɔ kekeakee nye be nàdi atikewɔwɔ tso eŋutinunyala bibi siwo nya SAID-tikewo ƒe nu sesẽwo nyuie gbɔ. Woate ŋu akpe ɖe viwòa ƒe dzesiwo dzi kpɔkpɔ ŋu eye woakpe ɖe eŋu wòakpɔ ɖevimenɔɣi nyuitɔ kekeake.

Nu vevitɔ kekeake siwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Enyo, eyata mina míaƒo nu tso nusiwo dzi wòle be nàɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me la dometɔ aɖewo ŋu kpuie.

• Ne asrã le viwòa ŋu enuenu, si womate ŋu aɖe o la , ƒo nu tso eŋu na ɖɔkta. Ate ŋu anye SAID ƒe dzesi.
• SAIDs nye dɔlélenutsiŋutete si wodzina ƒe kuxi , ke menye dɔléle si xɔa ame me si bɔ o.
• Esiawo to vovo tso ``Autoimmune'' dɔlélewo gbɔ .
• Zi geɖe la, domenyiŋusẽfianu gbɔe nusiawo tsona .
• Ele vevie be nàkpɔ Lãmetsiŋutinunyala be woakpɔ dɔlélea nyuie.
• Atikewɔwɔ ate ŋu aɖu dzesiawo dzi nyuie .
• Atikewɔwɔ kaba le vevie be woaxe mɔ ɖe ayiku ƒe gbegblẽ nu.

Ne nya bubu aɖewo gale asiwò ku ɖe esia ŋu la, mègavɔ̃ be yeabia wò ƒomea ƒe ɖɔkta alo ɖeviwo ƒe dɔdala o. Woate ŋu akpe ɖe ŋuwò geɖe wu.

` SAIDs, domenyiŋusẽfianu ƒe dzoxɔxɔ, dzoxɔxɔ enuenu, dzoxɔxɔ le ɖeviwo me, dɔlélenutsiŋutete, domenyiŋusẽfianu ƒe dɔlélewo, lãmekaŋutinunyala