So wo ba no taa nya atiridii? So ebetumi aba sɛ efi SAIDs (Systemic Autoinflammatory Diseases)?

Oh, so wo ba ketewa no taa nya atiridii nnansa yi? Bere a wususuw sɛ wo ho atɔ wo ara pɛ no, so ɛsan sɔ bio? Ɛtɔ da bi a, ebia nea ɛde eyi ba no nyɛ mmoawa anaa mmoawa a wɔtaa nya bi. Ɛnnɛ yɛbɛka tebea bi a ɛyɛ den kakra ho asɛm, nanso ɛho hia yiye sɛ yehu ho asɛm. Wɔfrɛ eyi SAIDs, a egyina hɔ ma Systemic Autoinflammatory Diseases . Bere bi a atwam no, na wɔfrɛ eyi `(Periodic Fever Syndromes)` anaa `(Recurrent Fever Syndromes)`.

Dɛn ne SAID ahorow? Momma yɛnte ase tiawa.

Sɛ yɛbɛka no tiawa a, SAID yɛ nyarewa kuw bi a yɛn nipadua mu nipadua mu ahoɔden a ɛko tia nyarewa a wɔde awo yɛn no ntumi nyɛ adwuma yiye anaasɛ mmara ho haw bi na ɛde ba . Eyi nti na abofra tumi nya atiridii mpɛn pii a ɔyare mmoawa (te sɛ mmoawa anaa mmoawa) mpo nni hɔ no.

Nea ɛho hia sen biara ne sɛ ɛnsɛ sɛ wɔde tebea horow a wɔfrɛ no SAIDs yi frafra `(Autoimmune Diseases)` (sɛ nhwɛso no `(Rheumatoid Arthritis)` anaa `(Lupus)` a ebia woate ho asɛm. Wɔ `(Autoimmune)` nyarewa mu no, esiane mfomso bi a ɛwɔ yɛn nipadua `(adaptive immune system)` mu nti, saa nhyehyɛe no tow hyɛ n’ankasa nkwammoaa a ɛwɔ apɔwmuden so. Nanso wɔ SAIDs mu no, biribi soronko bi si.

SAIDs ho yɛ na koraa sen nyarewa a nipadua no tumi ko tia nyarewa. Mpɛn pii no wonya fi awo mu na awosu mu nsakrae na ɛde ba .

Mpɛn pii no, nanso ɛnyɛ bere nyinaa na SAID sɛnkyerɛnne ahorow fi ase bere a abofra yɛ ketewaa koraa, sɛ́ akokoaa anaa abofra ketewaa . Ebia wo ba no benya "bere" a atiridii ne sɛnkyerɛnne afoforo a ɛwɔ saa tebea yi mu. Wɔ saa bere yi ntam no, ebia wo ba no rennya yare no ho sɛnkyerɛnne biara koraa. Ɛwom sɛ wonni SAIDs ano aduru de , nanso mpɛn pii no, ayaresa ahorow wɔ hɔ a ɛbɛboa ma woadi wo ba no yare no ho sɛnkyerɛnne ahorow so.

Dɛn ne SAID ahorow titiriw?

Nhwehwɛmufo ahu SAID ahorow bɛyɛ 60, na wɔda so ara rehu foforo. SAID ahorow a wɔtaa de di dwuma no bi ni:

• Abusua mu Mediterranea atiridii (FMF): Eyi ne atiridiinini a efi awosu mu a ɛsan ba bio no mu nea abu so sen biara. Ebetumi ama mmofra yafunu, wɔn koko, ne wɔn nkwaa ahonhon ma ɛyɛ yaw.
• Atiridiinini a ɛba bere ne bere mu, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Eyi taa fi ase wɔ mmofra nkumaa so ansa na wɔadi mfe 4. Ɛtɔ mmere bi a, tebea no ankasa betumi ayɛ yiye bere a wɔadi mfe 10 akyi.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Eyi betumi aba wɔ mmofraase, mmerantebere ne mmabaabere, anaa mpanyin afe so mpo.
• Mevalonate kinase deficiency (MKD): Kan no na wɔfrɛ no ``Hyper-IgD syndrome'', eyi taa fi ase ansa na abofra no adi afe biako.
• Nyarewa a ɛma obi ho hyehye no a ɛne NLRP3 wɔ abusuabɔ:Kan no na wɔfrɛ eyi `(Cryopyrin-associated periodic syndromes - CAPS)`. Subtypes afoforo abiɛsa wɔ eyi mu.
• Still yare a efi ase wɔ mpanyimfo mu (AOSD): Sɛnea edin no kyerɛ no, eyi yɛ mmofraberem tebea a efi ase wɔ mpanyimfo mu ``Systemic Juvenile Idiopathic Arthritis (JIA)``.
• NOD-2-associated granulomatous disease (Blau syndrome): Eyi taa fi ase ansa na wadi mfe 4. Ɛka abofra no honam ani, n’ani, ne ne nkwaa titiriw.

Dɛn ne SAIDs ho sɛnkyerɛnne?

SAIDs ho sɛnkyerɛnne taa fi ase wɔ mmofraase. Nsɛnkyerɛnne titiriw ne nea ɛtaa ba ne atiridii a ɛba bere ne bere mu . Wɔ atiridii bere mu no, mpɛn pii no, nkurɔfo tumi nya apɔwmuden na wonni yare no ho sɛnkyerɛnne biara. Nanso, ebia sɛnkyerɛnne afoforo bi wɔ hɔ a ɛfa SAID ahorow biara ho. Ma yɛnhwɛ nea wɔyɛ:

• FMF: Eyi betumi ama abofra no yafunu, ne koko, ne ne nkwaa ho ahonhon na ne ho ayɛ no yaw kɛse . Ebia akisikuru bɛba anan ase anaa anansoaa nso so.
• PFAPA: Eyi betumi ama ne menewa mu ayɛ no yaw, ano ayɛ kuru, na ntini a ɛwɔ kɔn mu a ahonhon . Sɛ nhwɛso no, sɛ wo ba no reda sɛnkyerɛnne ahorow te sɛ tonsillitis a ɛtaa ba ne n’ano akisikuru adi a, ebetumi ayɛ PFAPA.
• MFIA: Eyi betumi ama atiridii ne ntini ayɛ yaw wɔ dua/asen ne abasa mu . Ebetumi nso ama akisikuru kɔkɔɔ a ɛyɛ yaw a efi ase wɔ nsa ne nan so na ɛtrɛw kɔ nipadua no mu.
• MKD: Eyi betumi de sɛnkyerɛnne ahorow te sɛ atiridiinini, tiyɛ, yafunu mu yaw, aduan ho akɔnnɔ a ɛyera, ne sɛnkyerɛnne ahorow a ɛte sɛ atiridiinini aba .
• NLRP3 nyarewa: Saa tebea yi betumi ama honam ani ayɛ kurukuruwa, ti ayɛ no yaw, ayɛ basaa, nkwaa mu yaw, ne aniwa/aniwa a ɛyɛ pink .
• AOSD: Eyi yɛ tebea a ɛtaa ba a ɛma honam ani yɛ kurukuruwa, nkwaa mu yaw, ne ntini mu yaw . Nnipa binom nso betumi anya menewam yaw, yafunu mu yaw, ɔbrɛ, ne adwenemhaw.
• Blau syndrome: Eyi betumi ama wo ba no nsa, ne nan, anaa ne mfinimfini honam ani akuru . Ebetumi nso ama nkwaa ayɛ no yaw na aniwa ayɛ no yaw .

Dɛn na ɛde SAID ahorow ba?

SAID pii yɛ awosu mu nyarewa . Ɛne sɛ, nkwaadɔm mu nsakrae (variant) pɔtee bi a ɛwɔ awosu mu abɔde pɔtee bi mu na ɛde ɔyare biara ba. Fa no sɛ yɛn nipadua no wɔ akwankyerɛ (awosu mu nkwaadɔm) ahorow bi a ɛkyerɛ yɛn sɛnea yɛbɛyɛ adwuma biara, enti sɛ saa nhoma no mu nkyerɛwde biako yɛ mfomso a, ebetumi ama adwuma a aka no asɛe.

SAID ahorow atitiriw ne awosu mu nkwaadɔm a ɛbata ho ni:

• FMF: 1 .Gene `MEFV` ne nea ɛma akwankyerɛ wɔ sɛnea wɔyɛ protein `(pyrin)` no ho.
• PFAPA: Wɔnnya nhuu nea ɛde eyi ba ankasa .
• TRAPS: Awosu mu abɔde a nkwa wom `TNFRSF1A`. Eyi kyerɛ sɛ wɔnyɛ protein bi a wɔfrɛ no `(tumor necrosis factor receptor - TNFR)`.
• MDK: Awosu mu abɔde a wɔfrɛ no `MVK`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(mevalonic kinase)`.
• NLRP3 nyarewa: Awosu mu abɔde `NLRP3`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(cryopyrin)`.
• AOSD: Wɔnnya nhuu nea ɛde eyi ba ankasa .
• Blau syndrome: Awosu mu abɔde a nkwa wom `NOD2`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(NOD2)`.

Ɛho hia sɛ yɛte ase sɛ sɛ awosu mu nneɛma na ɛde eyinom ba a, ɛnyɛ awofo no mfomso. Eyinom yɛ abɔde mu nneɛma a ɛyɛ den yiye.

Nsɛnnennen a SAID ahorow de ba

Ɛho hia yiye sɛ wubenya ayaresa a ɛfata ama tebea horow yi. Efisɛ, sɛ ɔfe kɔ so wɔ nipadua no mu a, ebetumi de tebea bi a wɔfrɛ no `(Amyloidosis) aba. `(Amyloidosis)` yɛ protein a ɛwɔ asaabo mu . Eyi betumi asɛe asaabo no daa . Enti, sɛ sɛnkyerɛnne ahorow bi wɔ hɔ a, ɛho hia sɛ wohwehwɛ aduruyɛfo afotu ntɛm ara.

Ɔkwan bɛn so na wohu SAID ahorow?

Sɛ yɛbɛka nokware a, SAID ahorow a wobehu no betumi ayɛ den kakra efisɛ sɛnkyerɛnne ahorow no betumi ayɛ te sɛ tebea afoforo a emu yɛ den te sɛ lupus anaa lymphoma de. Enti, ɛho hia sɛ wokɔ oduruyɛfo a wanya ntetee titiriw wɔ nyarewa a ɛma obi ho hyehye no nkyɛn (oduruyɛfo a ɔyɛ akisikuru) na ama wahu wo ba no tebea no pɛpɛɛpɛ na wahwɛ so.

Wo ba no ntini ho ɔyaresafo de nneɛma pii bedi dwuma de ahu SAIDs. Ɔbɛbisa wo ba no yareɛ no ho nsɛnkyerɛnneɛ ne sɛ ebia obi wɔ w’abusua mu anya saa tebea yi (biological family history). Ebia oduruyɛfo no besusuw sɛ wo ba no wɔ Periodic Fever Syndrome sɛ:

• Sɛ wotaa nya atiridii a
• Sɛ obi wɔ abusua no mu a ɔwɔ abakɔsɛm a ɛkyerɛ sɛ ɔwɔ atiridiinini a ɛba bere ne bere mu te sɛ eyi a
• Sɛ woka nnipa kuw bi a wɔtaa nya atiridii a ɛte saa ho a

Sɔhwɛ ahorow bɛn na wɔde di dwuma?

Ebia wo duruyɛfo bɛkamfo akyerɛ sɛ wobɛyɛ nhwehwɛmu pii de akyerɛ sɛ woahu sɛ wowɔ yare no. Ebia eyinom bi ne:

• Lab nhwehwɛmu: Sɔhwɛ te sɛ `(C-reactive protein - CRP)` ne `(Complete Blood Count - CBC)` betumi ahwɛ sɛ ɔfe wɔ nipadua no mu anaa. Saa nhwehwɛmu yi yɛ `(positive)` bere a atiridiinini wɔ hɔ, na ɛsan kɔ ne tebea a ɛfata mu bere a atiridiinini no kɔ.
• Nsu mu nhwehwɛmu:Wobetumi ayɛ protein nhwehwɛmu wɔ nsu mu de ahwɛ sɛ protein pii wɔ nsu mu anaa. Wɔ MKD mu no, nsu mu nhwehwɛmu a wɔbɛyɛ de ahu organic acids bɛkyerɛ sɛ mevalonic acid dodow akɔ soro.
• Awosu mu nhwehwɛmu: Eyi betumi ahwɛ sɛ awosu mu nsakrae ahorow wɔ hɔ anaa. Nanso, ebia mmofra binom a wɔwɔ SAID no nni awosu mu nsakrae a wɔahu no. Enti, sɛ mpo sɔhwɛ no aba yɛ ``negative`` a, wontumi nnye ntom sɛ SAID betumi aba.

Ɔkwan bɛn so na wɔsa SAID ahorow?

Ɛsono sɛnea wɔsa SAID ahorow no gyina yare no su ne sɛnea emu yɛ den so . Ɛwom sɛ wontumi nsa nyarewa yi koraa de, nanso wobetumi adi abofra no sɛnkyerɛnne ahorow so yiye denam nnuru a wɔde di dwuma so . Sɛ nhwɛso no, sɛ wo ba nya atiridiinini yi mpɛn kakraa bi pɛ afe biara a, mpɛn pii no, nnuru a wɔde kum ɛyaw ne nnuru a ɛnyɛ steroid a wɔde kum ɔyare mmoawa (NSAID) betumi adi sɛnkyerɛnne ahorow no so. Nanso, sɛ yare no mu yɛ den kɛse a, oduruyɛfo no betumi akyerɛ ayaresa foforo a wobetumi afa so.

Ayaresa a ɛte saa no bi ni:

• FMF: Wobetumi de aduru bi a wɔfrɛ no `(Colchicine)` ayɛ eyi ho adwuma de atew ɔfe so. Sɛ woantumi annom `(Colchicine)` a, wobɛtumi asɔ `(Biologic)` aduru bi a wɔfrɛ no `(Canakinumab)` ahwɛ.
• PFAPA: Wobetumi atew PFAPA a ɛyɛ hyew no so denam steroids (a ɛtaa yɛ prednisone) a wɔde bɛma so. Mmofra binom atumi adi wɔn yare no ho sɛnkyerɛnne so denam cimetidine, aduru bi a wɔde sa yafunu mu akisikuru a wɔde di dwuma no so.
• TRAPS: Aduru `(Canakinumab)` no yɛ adwuma yiye ma TRAPS. Afei nso, nnuru a wɔkyerɛw ma ɛko tia ɔyare mmoawa te sɛ `(Glucocorticoids)` nso betumi aboa ma yare no ho sɛnkyerɛnne no afi hɔ.
• MKD: `(Canakinumab)` yɛ ɔkwan pa a wɔfa so sa MKD ayaresa. `(NSAIDs)` anaa `(Steroids)` a wode bedi dwuma bere a atiridiinini no nso betumi aboa.
• NLRP3 nyarewa: Wɔde nnuru afoforo ``Immunomodulators'' te sɛ ``Canakinumab'' ne ``Rilonacept'' ne ``Anakinra'' di dwuma yiye de sa NLRP3 nyarewa.
• AOSD: Wɔde nnuru ahorow a ekum ɔyare mmoawa di dwuma ma AOSD. Eyinom bi ne `(Steroids)`, `(Nnuru a Ɛko Tia Akisikuru a Ɛsakra Nyarewa - DMARDs)`, ne `(Biologics)`.
• Blau syndrome: Wobetumi asɔ nnuru a ɛma nipadua no tumi ko tia nyarewa, nnuru a esiw tumor necrosis factor (TNF) ano, ne/anaasɛ nnuru a wɔde gu aniwa so ahwɛ.

So SAIDs tebea no yɛ yiye wɔ n’ankasa so?

SAID ahorow bi betumi atra hɔ nkwa nna nyinaa . Ebia afoforo nso bɛtra hɔ mfe kakraa bi na sɛ obi nyin a, wɔn ho bɛtɔ wɔn. Bere a tebea horow bi di nkwa nna nyinaa no, atiridii a ɛtaa ba no so betumi atew bere a bere kɔ so no na yare no ho sɛnkyerɛnne ahorow no betumi ayɛ nea emu nyɛ den kɛse . Wo duruyɛfo betumi ama wo wo ba no tebea pɔtee no ho nsɛm pɔtee.

Abofra a ɔwɔ SAID a wɔbɛhwɛ no betumi ayɛ den. Ebia w’ankasa woaka, nanso wubetumi de w’ankasa osuahu ahorow aboa ma wɔahwɛ wo ba no na woaboa no ma wɔagyina tebea yi a ɛwɔ wɔn nkwa nna nyinaa mu ano.

Ade a ɛho hia sen biara ne sɛ wobɛhwehwɛ ayaresa afi nnuruyɛfo atitiriw a wonim SAID ahorow a ɛyɛ den no hɔ. Wobetumi aboa ma wɔadi wo ba no sɛnkyerɛnne ahorow ho dwuma na wɔaboa no ma wanya mmofraberem a eye sen biara.

Nneɛma a ɛho hia sen biara a ɛsɛ sɛ wokae (Take-Home Message) .

Okay, enti momma yɛmmɔ nneɛma bi a ɛsɛ sɛ wokae no mua mfi nea yɛaka ho asɛm no mu.

• Sɛ wo ba no taa nya atiridiinini a wontumi nkyerɛkyerɛ mu a , ka ho asɛm kyerɛ oduruyɛfo. Ebetumi ayɛ SAID ho sɛnkyerɛnne.
• SAIDs yɛ awosu mu nipadua mu ahoɔden a ɛko tia nyarewa no haw , ɛnyɛ yare a ɛtaa de ɔyare mmoawa ba.
• Eyinom yɛ soronko wɔ ``Autoimmune'' nyarewa ho .
• Mpɛn pii no, awosu mu nneɛma na ɛde eyinom ba .
• Ɛho hia sɛ wokɔ Rheumatologist nkyɛn na ama woahu yare no pɛpɛɛpɛ.
• Ayaresa betumi adi sɛnkyerɛnne ahorow no so yiye .
• Ɛho hia sɛ wɔsa yare ntɛm na ama asaabo asɛe.

Sɛ wowɔ nsɛmmisa foforo bi fa eyi ho a, nsuro sɛ wubebisa w’abusua oduruyɛfo anaa mmofra oduruyɛfo. Wɔbɛtumi aboa wo akɔ akyiri.

` SAIDs, atiridii a wonya fi awo mu, atiridii a ɛtaa ba, atiridii a ɛba mmofra mu, nipadua mu ahoɔden a ɛko tia nyarewa, awosu mu nyarewa, atiridiinini ho ɔbenfo