Ðe asrã si womenya o la dzea viwòa ŋu zi geɖea? Esia ate ŋu atso SAIDs (Systemic Autoinflammatory Diseases) gbɔ!

Ðe viwò suea xɔa asrã ɣesiaɣia? Ɣeaɖewoɣi la, woxɔa asrã zi ɖeka alo zi eve le ɣletia me, gake ɖɔktawo gblɔna be dɔlékui aɖeke, si fia be dɔlékui alo dɔlékui aɖeke mele ŋutilãa me o. Le ɣeyiɣi mawo me la, abe vidada alo vifofo ene la, susu le eme ŋutɔ be nàse vɔvɔ̃ kple dzimaɖitsitsi geɖe. "Nukatae esia le dzɔdzɔm ɖe vinye dzi?" Anɔ eme be èbu tame zi akpe ɖeka. Ðewohĩ nusitae wòle alea ɖo nye nusi ŋu mèse nu tsoe kpɔ o. Egbea, míaƒo nu tso SAID (Systemic Autoinflammatory Diseases), siwo gɔmesese sesẽ eye woate ŋu ahe asrã vɛ enuenu ŋu. Tsã la, woyɔa esiawo be `(Periodic Fever Syndromes)` alo `(Recurrent Fever Syndromes)`.

Nukae nye SAID (Systemic Autoinflammatory Diseases) siawo?

Kpuie ko la, SAID nye dɔlélewo ƒe hatsotso aɖe si dzɔna le míaƒe ŋutilã ƒe dzɔdzɔme dɔlélenutsiŋutete ƒe dɔmawɔmawɔ nyuie alo dziɖuɖu ƒe kuxi aɖe ta . Nusi dzɔna enye be, ne gotagome nusi gbɔ wòtso, si nye dɔlékui alo dɔlékui aɖeke manɔmee la, ɖeko ŋutilãa naa abi dona. Esia tae asrã sia si dona enuenu la vana ɖo.

Fifia ɖewohĩ ànɔ eŋu bum be, "Oo, ɖe esia nye Autoimmune Diseases mawo dometɔ ɖekaa?" Ao, ame eve siawo to vovo vie. Le kpɔɖeŋu me, le Autoimmune Diseases me la, le dɔlélewo abe Rheumatoid Arthritis alo Lupus ene me la, míaƒe ŋutilã ƒe dɔlélenutsiŋutete si 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 Ŋusẽna Le. Zi geɖe la, nusiawo nyea domenyinu , si fia be domenyiŋusẽfianu ƒe tɔtrɔ alo tɔtrɔ aɖe gbɔe wotso.

Zi geɖe la, SAID siawo dzea egɔme (gake menye ɣesiaɣie o) ne viwò nye vidzĩ alo ɖevi si le zɔzɔm . Ðevia akpɔ dɔlélea ƒe ɣeyiɣi aɖewo alo adze edzi le ɣeyiɣi aɖewo me. Le ɣeyiɣi siawo me la, asrãa kple dzesi bubuwo ate ŋu adze. Gake le "amedzidzedze" siawo dome la, ɖevia ate ŋu anye esi dzesi aɖeke manɔ eŋu o. Atike aɖeke meli na SAID-dɔlékuiwo o, gake zi geɖe la, atikewɔmɔnu nyuiwo li siwo dzi woato aɖu dzesiawo dzi.

Nukawoe nye SAID ƒomevi veviwo?

Numekulawo de dzesi SAID ƒomevi siwo ade 60, eye wole bubuwo dim. Wo dometɔ aɖewoe nye esiwo bɔ wu:

• Ƒomea ƒe Mediterranea-ƒua ƒe 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 navuvu vevesesetɔe.
• Dzoxɔxɔ si nɔa anyi ɣeaɖewoɣi, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Zi geɖe la, esia dzea egɔme tso ɖevi suewo gbɔ, zi geɖe hafi woxɔa ƒe 4. Gake le ɖevi aɖewo gome la, nɔnɔme sia ate ŋu nu ayi le ƒe 10 xɔxɔ megbe.
• Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS): Esia ate ŋu adzɔ tso ɖevime vaseɖe ƒewuiwo me, eye ɣeaɖewoɣi va ɖo ame tsitsi me.
• Mevalonate kinase ƒe anyimanɔmanɔ (MKD): Woyɔa esia tsã be Hyper-IgD syndrome, eye zi geɖe la, esia dzea egɔme hafi ɖevia xɔ ƒe ɖeka.
• 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): Abe alesi ŋkɔa ɖee fia ene la, dɔléle sia dzea egɔme tso ame tsitsi me. Enye ame tsitsiwo ƒe nɔnɔme si woyɔna be systemic juvenile idiopathic arthritis (JIA) ƒe ƒomevi.
• NOD-2-associated granulomatous disease (Blau syndrome): Zi geɖe la, esia hã dzea egɔme hafi wòxɔ ƒe 4. Edzea ɖevia ƒe ŋutigbalẽ, ŋku, kple ƒunukpeƒewo koŋ dzi.

Nukae nye SAID-dɔlékuiwo ƒe dzesiwo?

Zi geɖe la, SAID ƒe dzesiwo dzea egɔme tso ɖevimenɔɣi. Dzesi vevitɔ kple esi bɔ wue nye dzoxɔxɔ si nɔa anyi ɣeaɖewoɣi alo esi dzɔna ɣeaɖewoɣi . Abe alesi míegblɔe va yi ene la, ɖevia ate ŋu anɔ nyuie le ɣeyiɣi siwo me asrã aɖeke mele eŋu o me. Gake dzesi bubuwo li siwo ku ɖe SAID ƒomevi ɖesiaɖe koŋ ŋu:

• Le FMF me: Ðevia ƒe ƒodo, akɔta, kple ƒunukpeƒewo ate ŋu ase veve ŋutɔ eye wòavuvu. Ɣeaɖewoɣi la, ŋutigbalẽ ate ŋu axɔ afɔkpodzi alo afɔkpodzi.
• Le PFAPA me la: Kɔmeve, nu me abi, ʋuka siwo vuvu le kɔ me. Esiawoe nye dzesi vevitɔwo.
• Le TRAPS me: Ŋutilã ate ŋu azu vuvɔ eye asrã naxɔ. Ðevia ate ŋu anɔ veve sem le eƒe akɔta kple abɔwo me. Dzĩ aɖe si le veve sem ate ŋu ado, si ate ŋu akaka tso abɔwo dzi ayi afɔwo dzi eye emegbe ayi ŋutilã me.
• Le MKD me: Àte ŋu akpɔ dzesi siwo le abe dzamezã ene abe vuvɔ, taɖuame, dɔgbo ƒe veve, nuɖuɖu ƒe dzodzro, kple dzesi bubuwo ene.
• Le NLRP3 dɔlélewo me la: ŋutigbalẽ ƒe ʋuʋu, taɖuame, lãmetutudɔ, ƒunukpeƒeveame, kple ŋku ƒe ʋuʋu (ŋku ƒe dɔléle) ate ŋu adzɔ.
• Le AOSD me la: ŋutigbalẽ ƒe ʋuʋu, ƒunukpeƒeveame, lãmekawo ƒe vevesese. Ame aɖewo hã ate ŋu ase veve le ŋɔti me, dɔgbo nase veve, ɖeɖi tea wo ŋu, eye woase le wo ɖokui me be yewogbɔdzɔ.
• Le Blau ƒe dɔlélea me: Vidzĩa ate ŋu axɔ ŋutigbalẽ ƒe abi le abɔwo, afɔwo, alo titina. Woate ŋu ase ƒunukpeƒeveame kple ŋkumeve hã.

Bu eŋu kpɔ, Nalini vinyɔnuvi suea le asrã sesẽ lém ŋkeke etɔ̃ va ɖo ene ɣleti sia ɣleti ɣleti ʋɛ siwo va yi. Tsɔ kpe ɖe ema ŋu la, abi suesuesuewo hã le eƒe nu me kple ƒunukpeƒewo le eƒe kɔ ŋu. Esi wòtsɔe fia ɖɔkta la, egblɔ be ate ŋu anye PFAPA.

Nukawoe hea SAID-dɔlékuiwo vɛ?

SAID akpa gãtɔ nye domenyiŋusẽfianu ƒe dɔlélewo . Esia fia be míaƒe domenyiŋusẽfianu ƒe tɔtrɔ gbɔe dɔléle siawo dometɔ ɖesiaɖe tsona. SAID ƒomevi siwo bɔ wu kple domenyiŋusẽfianu siwo gblẽa nu le wo ŋue nye esi:

• FMF: Domenyiŋusẽfianu si woyɔna be `(MEFV)`. Domenyiŋusẽfianu sia fiaa mɔ protein `(pyrin)` ƒe wɔwɔ.
• PFAPA: Womekpɔ de dzesi domenyiŋusẽfianu si tututu kpɔa ŋusẽ ɖe esia dzi haɖe o.
• TRAPS: Domenyiŋusẽfianu si woyɔna be `(TNFRSF1A)`. Afi siae mɔfiame siwo ku ɖe protein `(tumor necrosis factor receptor - TNFR)` wɔwɔ ŋu tso.
• MKD: Domenyiŋusẽfianu si nye `(MVK)`. Esia fiaa mɔ protein si woyɔna be `(mevalonic kinase)` ƒe wɔwɔ.
• NLRP3 dɔlélewo: Domenyiŋusẽfianu si woyɔna be `(NLRP3)`. Esia naa mɔfiamewo le protein si woyɔna be `(cryopyrin)` wɔwɔ ŋu.
• AOSD: Womenya nusi tututu tae wòle alea ɖo haɖe o.
• Blau ƒe dɔléle: Domenyiŋusẽfianu si nye `(NOD2)`. Esia fiaa mɔ protein `(NOD2)` ƒe wɔwɔ.

Domenyiŋusẽfianu siawo ŋuti nyatakakawo adze na wò be wosesẽ vie. Kpuie ko la, domenyiŋusẽfianu siawo kpɔa ŋusẽ ɖe protein aɖewo siwo kpɔa dɔdzẽ dzi le míaƒe ŋutilã me la wɔwɔ dzi. Esia tae ŋutilãa hea dɔdzẽ ƒe nɔnɔmewo vɛ le susu aɖeke manɔmee.

Nukae ate ŋu adzɔ ne womewɔ atike na SAID-wo nyuie o?

Ele vevie ŋutɔ be woakpɔ atike nyui na SAID siawo. Elabena ne dɔdzẽa yi edzi le ŋutilãa me la, nɔnɔme aɖe si woyɔna be `(amyloidosis)` ate ŋu ado mo ɖa. Esia fia be protein ƒomevi aɖe nɔa míaƒe ayikuwo me, si ate ŋu agblẽ nu le ayikuwo ŋu tegbee . Eyata ne dzesi aɖewo li la, ele vevie ŋutɔ be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo evɔ màŋe aɖaba aƒu wo dzi o.

Aleke ɖɔktawo wɔa dea dzesi SAID-dɔlékuiwoe?

SAID-dɔlékuiwo didi ate ŋu asesẽ elabena dzesiawo ate ŋu asɔ kple dɔléle sesẽ bubuwo abe lupus alo ʋumenugbagbeviɣidɔ ene. Eyata , anyo wu be nàkpɔ ƒunukpeƒeŋutinunyala, ɖɔkta si xɔ hehe tɔxɛ le dɔdzẽ ƒe dɔlélewo ŋu , be wòakpɔ viwòa ƒe nɔnɔmea nyuie ahakpɔ edzi.

Viwò ƒe lãmekaŋutinunyala alé ŋku ɖe nu geɖe ŋu atsɔ anya SAID-dɔlékuiwo. Abia nya wò tso viwòa ƒe dzesiwo kple nenye be ame aɖe si ŋu dzamezã le enuenu le wò ƒomea me ŋu. Ðɔktaa ate ŋu asusui be nɔnɔme sia le eŋu ne:

• Ne asrã le ɖevia ŋu enuenu la .
• Ne ƒomea me tɔ aɖe xɔ asrã enuenu kpɔ .
• Ne ɖevia tso to aɖe si me asrãdɔ mawo ate ŋu adze enuenu wu la me .

Dodokpɔ kawoe wozãna?

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

• Lab dodokpɔwo: Ʋudodokpɔwo abe `(C-reactive protein - CRP)` kple `(Complete blood count - CBC)` ateŋu akpɔe ɖa be dɔdzẽ le ŋutilãa me hã. Dodokpɔ siawo nye `(positive)` ne asrã le ŋuwò eye wògatrɔna va ɖoa nɔnɔme nyuitɔ me ne asrãa nu fa.
• Aɖuɖɔ dodokpɔ: Woléa ŋku ɖe aɖuɖɔa me be protein sɔ gbɔ ɖe eme hã. Le MKD ƒe nɔnɔmewo me la, wogakpɔa aɖuɖɔa me be organic acid si woyɔna be ``mevalonic acid'' sɔ gbɔ ɖe eme hã.
• Domenyiŋusẽfianu dodokpɔ: Domenyiŋusẽfianu dodokpɔ ate ŋu alé ŋku ɖe domenyiŋusẽfianu ƒe vovototo siwo míeyɔ ɖe etame ŋu. Gake ɖewohĩ domenyiŋusẽfianu ƒe tɔtrɔ siwo ŋu woke ɖo la manɔ ɖevi aɖewo siwo ŋu SAID le ŋu o, eyata dodokpɔa me tsonu ate ŋu anye be vɔ̃.

Nukae nye atike siwo wozãna na SAID-dɔlékuiwo?

SAID-dɔlékuiwo dada nɔ te ɖe dɔlélea ƒomevi kple eƒe sesẽ dzi . Atike aɖeke meli na nɔnɔme siawo o, gake atikewo ate ŋu akpe ɖe viwòa ƒe dɔlélea ƒe dzesiwo dzi ɖuɖu ŋu . Ne viwòa dzea viwòa dzi zi ʋɛ aɖewo ko le ƒea me la, zi geɖe la, àte ŋu atsɔ vevesesenutsitikewo kple atike siwo me steroid mele o (NSAID) akpɔ dzesiawo gbɔ. Gake ne dɔlélea nu sẽ wu la, wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàzã atike bubuwo:

• FMF: Woateŋu atsɔ atike si woyɔna be `(Colchicine)` ada esia be wòaɖe abi dzi akpɔtɔ. Ne womateŋu ana `(Colchicine)` ɖevia o la, àteŋu ate atike ƒomevi aɖe si woyɔna be `(Canakinumab)` kpɔ.
• PFAPA: Steroidwo abe prednisone ene ate ŋu aɖe PFAPA ƒe "amedzidzedze" ƒe ɣeyiɣi didi dzi akpɔtɔ. Cimetidine, si nye atike si wonaa ɖevi aɖewo ɖe dɔgbo ƒe abi ta hã ate ŋu akpe ɖe ame ŋu wòaɖe dzesiawo dzi akpɔtɔ.
• TRAPS: Atike si nye `(Canakinumab)` wɔa dɔ ŋutɔ na TRAPS. Azɔ hã, woate ŋu atsɔ atike siwo tsia dzodzodzoetsitsi nu abe `(glucocorticoids)` siwo ɖɔkta ŋlɔ na la aɖu dzesiawo dzi.
• MKD: `(Canakinumab)` hã ​​nye atike nyui aɖe na MKD. `(NSAIDs)` alo `(steroids)` ateŋu akpeɖeŋu le "amedzidzedze" me.
• NLRP3 dɔlélewo: Wozã dɔlélenutsiŋutete ƒe atikewo abe Canakinumab, Rilonacept, kple Anakinra ene dzidzedzetɔe tsɔ da dɔléle siwo do ƒome kple NLRP3.
• AOSD: Wozãa atike vovovo siwo tsia dɔdzẽ nu abe steroid, atike siwo trɔa dɔléle siwo tsia lãmekawo nu (DMARDs), kple dɔlékuiwutikewo tsɔ daa AOSD.
• 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.

Nuxexlẽ tso atike siawo ŋu ate ŋu ana vɔvɔ̃ naɖo ŋuwò vie. Gake ɖo ŋku edzi be wotsɔa atike siawo katã naa wo le ɖɔkta ƒe dzikpɔkpɔ te vevie, le mɔ si asɔ na ɖevia wu nu. Eyata ele vevie be nàwɔ ɖe atikewɔwɔa dzi pɛpɛpɛ abe alesi ɖɔkta gblɔe ene.

Ðe SAIDs ƒe nɔnɔme le yiyima?

SAID aɖewo nye nɔnɔme siwo nɔa anyi le ame ƒe agbemeŋkekewo katã me . 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 ate ŋu aɖe akpɔtɔ eye dzesiawo magasẽ ɖe edzi o. Wò ɖɔkta aɖe viwòa ƒe nɔnɔmea koŋ me na wò.

Ðevi si ŋu SAID le dzi kpɔkpɔ ate ŋu asesẽ. Ne wò ŋutɔ nègblɔe la, àte ŋu azã wò nuteƒekpɔkpɔ atsɔ akpe asi ɖe viwòa dzi kpɔkpɔ ŋu. Wò nugɔmesese le vevie hã le kpekpe ɖe viwòa ŋu be wòanɔ agbe ɖe nɔnɔme sia si le eƒe agbemeŋkekewo katã me la nu.

Nu vevitɔe nye be nàdi atike tso ɖɔkta bibi siwo nya nu tso SAID-dɔlékuiwo ŋu 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 míagbugbɔ aƒo nu tso nya vevi aɖewo siwo dzi wòle be nàɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me ŋu:

• Ne asrã le viwòa ŋu enuenu, si womate ŋu aɖe o la , ate ŋu anye SAID ƒe dzesi.
• Menye dɔlékui aɖe gbɔe SAID-wo tsona o , ke boŋ enye kuxi si le ŋutilãa ŋutɔ ƒe dɔlélenutsiŋutete ŋu.
• Esiawo nye dɔléle siwo mebɔ o siwo domenyiŋusẽfianu gbɔe wòtsona zi geɖe .
• Dzesiawo ate ŋu ato vovo le SAID ƒe ƒomevi nu, gake dzoxɔxɔ enuenue nye dzesi vevitɔ .
• Ele vevie be nàyi Lãmetsiŋutinunyala gbɔ be woakpɔ dɔlélea nyuie ahadae.
• Togbɔ be atikewɔwɔ mate ŋu ada dɔlélea keŋkeŋ o hã la, woate ŋu aɖu dzesiawo dzi nyuie .
• Mègaŋe aɖaba ƒu dzesiawo dzi o, elabena ate ŋu ana ayiku nagblẽ ne womewɔ atike nɛ o.

Ne nyabiase bubu aɖewo gale asiwò ku ɖe esia ŋu la, mègahe ɖe megbe le nuƒoƒo na wò ƒomea ƒe ɖɔkta alo ƒunukpeƒeŋutinunyala o. Woate ŋu akpe ɖe ŋuwò geɖe wu.

` SAIDs, dzoxɔxɔ ƒe dɔléle siwo dzɔna ɣeaɖewoɣi, dzoxɔxɔ si gbugbɔna, autoinflammatory dɔlélewo, dzoxɔxɔ, dzoxɔxɔ le ɖeviwo me, domenyiŋusẽfianu ƒe dɔlélewo