Ðe asrã dzea viwòa dzi zi geɖe le susu manɔmeea? Mina míasrɔ̃ nu tso SAID (Systemic Autoinflammatory Diseases) siawo ŋu!

Ðe viwò suea xɔa asrã ko ɣeaɖewoɣi dzamezã alo dɔléle bubu aɖeke manɔmeea? Ðe asrãa va, nu fa le ŋkeke ʋɛ aɖewo megbe, eye wògadzena le ŋkeke ʋɛ aɖewo megbea? Dzila aɖewo doa go nuteƒekpɔkpɔ mawo tɔgbe. Eyata egbea míele nu ƒom tso nusi hea asrã vɛ enuenu, si didi sesẽ alea ŋu. Woyɔa esia be SAIDs (Dɔléle Siwo Me Dɔléle Siwo Wotsɔna Ða Ŋui le Ðoɖo Nu) . Tsã la, woyɔa esia be `(Periodic Fever Syndromes)` alo `(Recurrent Fever Syndromes)`.

Nukae nye SAID siawo? 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 . Le go sia me la, ne gotagome dɔlékui aɖeke (abe dɔlékui alo dɔlékui ene) mele míaƒe ŋutilã me o la, ɖeko míaƒe ŋutilã nana míexɔa dɔdzẽ. Ðeko wòle abe ɖe míaƒe ŋutilã ƒe ametakpɔnu le ʋukuku me wògbɔa eme ene.

Bu eŋu kpɔ, asrafowo ƒe ƒuƒoƒo aɖe le míaƒe ŋutilã me (emae nye dɔlélenutsiŋutete). Woƒe dɔe nye be woawɔ avu kple dɔlékui siwo hea dɔléle vɛ ne woge ɖe eme Gake le amesi ŋu SAID le ƒe ŋutilã me la, ɖeko asrafo siawo tsia dzodzodzoe ɣeaɖewoɣi eye wohea kuxiwo vɛ le ŋutilãa me.

Fifia àte ŋu anɔ ɖokuiwò biam be ɖe esia nye nane abe `(autoimmune diseases)` (si nye autoimmune diseases). Le kpɔɖeŋu me, `(ƒunukpeƒetetedɔ)` alo `(lupus)` sɔ kple wo nɔewo. Ao, ame eve siawo to vovo vie. Le dɔléle siwo dɔlélenutsiŋutete wɔna me la, nusi dzɔnae nye be míaƒe ŋutilã ƒe dɔlélenutsiŋutete si woxɔ alo esi trɔna ɖe nɔnɔmewo ŋu la dzea eya ŋutɔ ƒe lãmenugbagbevi siwo le lãmesẽ me dzi vodadatɔe. Gake le SAID-wo me la, dɔlélea ƒe dɔlélenutsiŋutete si wodzina la gbɔe kuxia le.

SAID-tikewo mebɔ kura o wu dɔléle siwo dɔlélenutsiŋutete hena vɛ. Wo dometɔ akpa gãtɔ nye domenyiŋusẽfianu , si fia be domenyiŋusẽfianu ƒe tɔtrɔ alo tɔtrɔ aɖe gbɔe wotso.

Zi geɖe la , SAIDs dzea egɔme tso vidzĩme alo ɖevimenɔɣi . Gake menye nenemae wònɔna ɣesiaɣi o. Asrã kple dzesi bubuwo ate ŋu anɔ viwòa ŋu ɣeaɖewoɣi alo adze edzi. Ðewohĩ dzesi aɖeke manɔ viwòa ŋu kura le amedzidzedzeawo dome o. Atike aɖeke meli na SAID-dɔlékuiwo o. Gake atikewɔmɔnu aɖewo li siwo ate ŋu akpe ɖe ame ŋu wòaɖu dzesiawo dzi le go akpa gãtɔ me.

Nukawoe nye SAID ƒomevi veviwo?

Fifia numekulawo de dzesi SAID ƒomevi siwo ade 60, eye wole bubuwo dim. Mina míalé ŋku ɖe ƒomevi siwo bɔ wu dometɔ aɖewo ŋu.

• Ƒomea ƒe Mediterranea Dzoxɔxɔ (FMF): Esiae nye dɔléle siwo bɔ wu le domenyiŋusẽfianu ƒe dɔléle siwo gbugbɔna dzɔna la dome. Ate ŋu ana ɖevia ƒe ƒodo, akɔta, kple ƒunukpeƒewo naxɔ abi vevesesetɔe .
• Dzoxɔxɔ si nɔa anyi ɣeaɖewoɣi, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Zi geɖe la, esia dzea egɔme tso ɖevi suewo dzi hafi woxɔa ƒe 4. Nɔnɔme sia ate ŋu aɖiɖi le eɖokui si le ƒe 10 xɔxɔ vɔ megbe.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Esia ate ŋu adzɔ le ɖevime, ƒewuiwo, kple ɣeaɖewoɣi le ame tsitsi gɔ̃ hã me.
• Mevalonate kinase ƒe anyimanɔmanɔ (MKD): Woyɔa esia tsã be hyper-IgD syndrome, eye zi geɖe la, esia dzea egɔme tso vidzĩ siwo mexɔ ƒe 1 haɖe o dzi.
• Dɔléle siwo do ƒome kple NLRP3: Tsã la, woyɔa esia be cryopyrin-associated periodic syndromes (CAPS). Ƒomevi sue etɔ̃ bubuwo hã le esia me.
• Still ƒe dɔléle si dzea egɔme tso ame tsitsiwo me (AOSD): Esia nye ɖeviwo ƒe ƒunukpeƒetetedɔ (JIA) si dzea egɔme tso ame tsitsiwo gbɔ.
• 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 dzi .

Nukae nye SAID-dɔlékuiwo ƒe dzesiwo? Aleke míewɔna dea dzesi woe?

Zi geɖe la, SAID ƒe dzesiwo dzea egɔme tso ɖevime. Dzesi si bɔ wu eye wòɖe dzesi wue nye dzoxɔxɔ si nɔa anyi ɣeaɖewoɣi alo esi dzɔna ɣeaɖewoɣi . Zi geɖe la, amewo nɔa anyi dzesi aɖeke manɔmee le amedzidzedzewo dome. Gake dzesi bubuwo ate ŋu anɔ anyi siwo ku ɖe SAID ƒomevi ɖesiaɖe koŋ ŋu.

Mina míalé ŋku ɖe SAID ƒomevi geɖe siwo ŋu míeƒo nu tsoe le etame ƒe dzesi tɔxɛwo ŋu:

• FMF: Esia ate ŋu ana ɖevia nase veve vevie le eƒe ƒodo, akɔta, kple eƒe ƒunukpeƒewo, tsɔ kpe ɖe dɔdzẽ ŋu . Ɣeaɖewoɣi la, ʋuʋudedi ate ŋu adze afɔ siwo le ete alo afɔkpodziwo dzi. Bu eŋu kpɔ, kasia ɖevi aɖe ƒe dɔgbo le vevem eye wòxɔa asrã. Ne dzilawo tsi dzi eye wokplɔ wo yi kɔdzi la, woate ŋu abui be ƒunukpeƒetetedɔe. Gake emegbe la, eɖea dzi ɖi eye wògatrɔna gbɔna le ŋkeke ʋɛ aɖewo megbe.
• PFAPA: Esia ate ŋu ahe dzesiwo abe ŋɔti me veve, nu me abi, kple ʋuka siwo vuvu le kɔ me vɛ .
• MOTƆWO: Esia ate ŋu ana ame natsi dzodzodzoe, lãmekawo nase veve le akɔta kple abɔwo me , eye ʋuʋudedi dzĩ si le veve sem si dzea egɔme tso abɔwo kple afɔwo dzi eye wòkakana ɖe ŋutilã bliboa me.
• MKD: Le esia hã meÐewohĩ màse le ɖokuiwò me be yeƒe lãme mekɔ o ne vuvɔ le wɔwɔm, taɖuame, dɔgbo ƒe veve, nuɖuɖu megadzroa wò o, kple dzesi siwo le abe dzamezã ene.
• NLRP3 dɔlélewo: Esiawo ate ŋu ahe dzesiwo abe ŋutigbalẽ ƒe ʋuʋu, taɖuame, lãmetutudɔ, ƒunukpeƒeveame, kple ŋku biã (ŋku ƒe ʋuʋu) vɛ .
• AOSD: Esia nye dɔléle si nana ŋutigbalẽ ƒe ʋuʋudedi, ƒunukpeƒeveame, kple lãmekawo ƒe vevesese zi geɖe . Ame aɖewo hã ate ŋu ase veve le ŋɔti me, dɔgbo nave wo, ɖeɖi tea wo ŋu vevie, eye woƒe ŋutilã nave wo.
• Blau ƒe dɔléle: Esia ate ŋu ana ŋutigbalẽ naxɔ viwòa ƒe abɔwo, afɔwo, alo ŋutilã . Ate ŋu ana ƒunukpeƒewo nase veve eye ŋku nase veve hã .

Nukatae SAID siawo dzɔna? Nukawoe gbɔ wòtsona?

SAID akpa gãtɔ nye domenyiŋusẽfianu ƒe dɔlélewo . Eyae nye be domenyiŋusẽfianu aɖe koŋ ƒe tɔtrɔ (to vovo) gbɔe dɔléle ɖesiaɖe tsona.

Mina míalé ŋku ɖe domenyiŋusẽfianu siwo kpɔa ŋusẽ ɖe SAID ƒomevi vevi geɖe dzi ŋu:

• FMF: Esia tsoa domenyiŋusẽfianu si woyɔna be `(MEFV domenyiŋusẽfianu)` gbɔ. Domenyiŋusẽfianu sia fiaa alesi woawɔ protein si woyɔna be `(pyrin)`.
• PFAPA: Womeke ɖe susu si tututu tae wòle alea ɖo ŋu haɖe o .
• TRAPS: Domenyiŋusẽfianu si woyɔna be `(TNFRSF1A gene)` gbɔe esia tsona. Esiae naa mɔfiamewo hena protein si woyɔna be `(tumor necrosis factor receptor - TNFR)` wɔwɔ.
• MKD: Nusitae nye domenyiŋusẽfianu si woyɔna be `(MVK domenyiŋusẽfianu)`. Esia fiaa mɔ protein si woyɔna be `(mevalonic kinase)` ƒe wɔwɔ.
• NLRP3 dɔlélewo: Esia tsoa domenyiŋusẽfianu si woyɔna be `(NLRP3 domenyiŋusẽfianu)` gbɔ. Esia naa mɔfiame be woawɔ protein si woyɔna be `(cryopyrin)`.
• AOSD: Womeke ɖe susu si tututu tae wòle alea ɖo ŋu haɖe o .
• Blau ƒe dɔléle: Domenyiŋusẽfianu si woyɔna be `(NOD2 domenyiŋusẽfianu)` gbɔe esia tsona. Esia fiaa mɔ protein si woyɔna be `(NOD2)` ƒe wɔwɔ.

Ðe SAID siawo ate ŋu ahe kuxi bubuwo vɛa?

Ẽ, esia ate ŋu ahe kuxiwo vɛ ne womewɔ atike nɛ nyuie o . Ne dɔdzẽ yi edzi le ŋutilã me la, ateŋu ahe nɔnɔme aɖe si woyɔna be ``amyloidosis.`` Eyae nye be protein ƒomevi aɖe nɔa míaƒe ayikuwo me. Esia ate ŋu agblẽ nu le ayikuawo ŋu tegbee . Esia tae wòle vevie be woanya dɔlélea kaba eye woakpɔ atike nɛ.

Aleke ɖɔkta wɔa anya SAIDs ƒe dɔléle siae?

Le nyateƒe me la, dɔléle siawo siwo ame ŋutɔ ƒe dɔlékuiwo ʋãna la didi ate ŋu asesẽ vie , elabena woƒe dzesiwo ate ŋu asrɔ̃ nɔnɔme sesẽ bubuwo abe lupus alo kansa abe ʋumenugbagbeviɣidɔ ene. Eyata ele vevie be nàkpɔ ɖɔkta si xɔ hehe tɔxɛ le dɔdzẽ ƒe dɔlélewo ŋu, si nye lãmekaŋutinunyala, be wòakpɔ viwò ƒe nɔnɔmea nyuie ahakpɔ edzi.

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

• Ne asrã le ɖevia ŋu enuenu la .
• Ne ƒomea me tɔ aɖe xɔ asrã enuenu abe esia ene kpɔ .
• Ne ɖevia le amewo ƒe ƒuƒoƒo aɖe si te ŋu xɔa dzoxɔxɔ ɣeaɖewoɣi abe esia ene me .

Dodokpɔ kawoe wole wɔwɔm na esia?

Be viwòa ƒe lãmekaŋutinunyala ate ŋu aɖo kpe dɔlélea dzi la, ate ŋu aɖo aɖaŋu be nàwɔ dodokpɔ geɖe. Esiawo ate ŋu anye:

• Lab dodokpɔwo: Woateŋu azã dodokpɔwo abe ``C-reactive protein (CRP)`` kple ``complete blood count (CBC)`` atsɔ akpɔe ɖa be dɔdzẽ le ŋutilã me hã. Dodokpɔ siawo nyea dɔlékuiwo le dzoxɔxɔ ƒe "amedzidzedze" me eye wogatrɔna yia nɔnɔme si sɔ me ne "amedzidzedze" la wu enu.
• Aɖuɖɔtoe dodokpɔ: Woate ŋu awɔ aɖuɖɔ me protein dodokpɔ atsɔ akpɔe ɖa be protein si sɔ gbɔ ɖe aɖuɖɔ me hã. Le MKD me la, aɖuɖɔ dodokpɔ si me woléa ŋku ɖe organic acid (acid siwo me carbon atɔm ɖeka le) ŋu ate ŋu aɖe mevalonic acid ƒe agbɔsɔsɔ si dzi ɖe edzi afia.
• Domenyiŋusẽfianu dodokpɔ: Esia ate ŋu alé ŋku ɖe domenyiŋusẽfianu ƒe vovototowo ŋu. Gake ɖewohĩ domenyiŋusẽfianu ƒe vovototo siwo ŋu woke ɖo la manɔ ɖevi aɖewo siwo ŋu SAID le la si o, si ate ŋu ana dodokpɔa me tsonu madze o.

Nukae nye atike siwo wozãna na SAID siawo?

Atikewɔwɔ na SAIDs toa vovo le nɔnɔmea ƒomevi kple eƒe sesẽ nu . Atike aɖeke meli na nɔnɔme siawo o. Gake zi geɖe la , woate ŋu atsɔ atikewo aɖu viwòa ƒe dzesiwo dzi . Ne viwòa dzea viwòa dzi zi ʋɛ aɖewo ko le ƒea me la, àte ŋu ana vevesesenutsitikewo abe nonsteroidal anti-inflammatory drugs (NSAIDs) ene be wòakpe ɖe eŋu wòaɖe woƒe dzesiwo dzi akpɔtɔ. Gake ne dɔlélea nu sẽ wu la, wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàzã atikewɔmɔnu bubuwo.

Wo dometɔ aɖewoe nye esi:

• FMF: Woateŋu azã atike si woyɔna be `(colchicine)` atsɔ aɖu esia dzi. Esia ɖea dɔdzẽ dzi kpɔtɔna. Ne ɖevia mete ŋu no colchicine o la, woate ŋu ate atike ƒomevi aɖe si woyɔna be `(canakinumab)` kpɔ.
• PFAPA: Woate ŋu ana steroid (zi geɖe la, prednisone) be woatsɔ aɖe PFAPA ƒe "amedzidzedze" ƒe ɣeyiɣi didi dzi akpɔtɔ. Le ɖevi aɖewo gome la, cimetidine, si nye atike si wozãna tsɔ daa dɔgbo ƒe abi, hã ate ŋu akpe ɖe ame ŋu woaɖu dzesiawo dzi.
• MOTƆWO: Atike si woyɔna be `(canakinumab)` wɔa dɔ nyuie na esia zi geɖe. Azɔ hã, woate ŋu aɖe dzesiawo dzi akpɔtɔ to atike siwo tsia dzodzodzoetsitsi nu siwo ɖɔkta ŋlɔ na wo, abe `(glucocorticoids)` ene.
• MKD:Canakinumab hã nye atike nyui aɖe na MKD. NSAID alo steroid nana le "amedzidzedze" me hã ateŋu akpeɖeŋu.
• NLRP3 dɔlélewo: Wozãa dɔlélenutsiŋutete ƒe atikewo abe canakinumab, rilonacept, kple anakinra ene dzidzedzetɔe na esia.
• AOSD: Wozãa atike vovovo siwo tsia dzodzodzoetsitsi nu na AOSD. Kpɔɖeŋu aɖewoe nye steroid-tikewo, atike siwo trɔa asi le dɔléle ŋu (DMARDs), kple dɔlékuiwutikewo.
• Blau ƒe dɔléle: Le viwòa ƒe dzesiwo nu la, ate ŋu ate dɔlélenutsiŋutetenutsitikewo, tumor necrosis factor (TNF) inhibitors, kple/alo ŋkutike siwo wozãna ɖe ŋku dzi la kpɔ.

Ðe SAIDs ƒe nɔnɔme siawo nɔa anyi ɖaa? Alo ɖe woadzoa?

Autoinflammatory nɔnɔme aɖewo nye agbemeŋkekewo katã ƒe nɔnɔmewo . Bubuwo ate ŋu anɔ anyi ƒe ʋɛ aɖewo eye woadzo ne wole tsitsim. Nɔnɔme aɖewo nɔa ame ƒe agbemeŋkekewo katã me, gake le ɣeyiɣi aɖe megbe la, amedzidzedze zi gbɔ zi geɖe kple woƒe sesẽme ate ŋu aɖe akpɔtɔ. Viwò ƒe ɖɔkta ate ŋu ana nyatakaka tɔxɛwo wò tso viwòa ƒe nɔnɔmea ŋu.

Ðevi si ŋu autoinflammatory dɔléle le hehe ate ŋu asesẽ. Ne SAID le asiwò la, àte ŋu azã wò ŋutɔ wò nuteƒekpɔkpɔwo atsɔ ado alɔ viwòa dzikpɔkpɔ nyuie wu. Àte ŋu azã wò nuteƒekpɔkpɔwo hã atsɔ akpe ɖe viwòa ŋu wòatrɔ ɖe nɔnɔme sia ŋu le eƒe agbemeŋkekewo katã me.

Nu vevitɔe nye be nàdi atikewɔwɔ tso eŋutinunyala bibi siwo nya nu tso SAID-dɔlékuiwo ŋu gbɔ. Ðɔkta mawo ate ŋu akpe ɖe viwòa ƒe dzesiwo dzi kpɔkpɔ ŋu eye woakpe ɖe eŋu wòakpɔ ɖevimenɔɣi nyuitɔ kekeake.

Gbedasi Mamlɛtɔ si Wotsɔ Yi Aƒeme

Eyata, mele mɔ kpɔm be susu aɖe le asiwò le SAID siwo ŋu míeƒo nu tsoe egbea ŋu. Ðo ŋku edzi be ne asrã le viwòa ŋu enuenu, si womate ŋu aɖe o la, ƒo nu tso eŋu na ɖɔkta godoo . Mègaŋe aɖaba ƒu edzi ko, asusu be esɔ o.

• SAIDs nye dɔlélewo ƒe hatsotso aɖe si hea asrã vɛ zi geɖe eye dɔlélenutsiŋutete ƒe kuxi aɖe gbɔe wòtsona.
• Zi geɖe la, nusiawo nyea domenyiŋusẽfianu eye wodzea egɔme tso woƒe ɖevime.
• Togbɔ be atike aɖeke meli na esia tegbee o hã la, atikewɔmɔnu aɖewo li siwo ate ŋu aɖu dzesiawo dzi.
• Ele vevie be woadi dɔlélea didi nyuie ahadae tso ɖɔkta bibi aɖe gbɔ (Rheumatologist).
• Ne wodze atikewɔwɔ gɔme kaba la, woate ŋu axe mɔ ɖe kuxiwo abe amyloidosis ene nu eye ɖevia ate ŋu anɔ agbe si sɔ.

Ne nya bubu aɖewo gale asiwò ku ɖe esia ŋu la, mègahe ɖe megbe le wò ƒomea ƒe ɖɔkta alo ɖeviwo ƒe dɔdala biabia me o.

` SAIDs, Systemic Autoinflammatory Diseases, Dzoxɔxɔ Enuenu, Domenyiŋusẽfianu ƒe Dɔlélewo, Dzoxɔxɔ le Ðeviwo me, Dɔlélenutsiŋutete, Lãmetsiŋutinunyala