So wo ba no taa nya atiridii a wontumi nkyerɛkyerɛ mu? Eyi betumi afi SAIDs (Systemic Autoinflammatory Diseases) ho!

So wo ba kumaa no nya atiridii bere nyinaa? Ɛtɔ da a, wonya atiridii pɛnkoro anaa mprenu ɔsram biara, nanso nnuruyɛfo no ka sɛ ɔyare mmoawa biara nni nipadua no mu, kyerɛ sɛ, mmoawa anaa mmoawa biara nni nipadua no mu. Wɔ mmere a ɛtete saa mu no, sɛ ɛna anaa agya no, ntease wom paa sɛ wobɛte ehu ne dadwen pii nka. "Adɛn nti na eyi rekɔ so wɔ me ba no so?" Ebia woasusuw ho mpɛn apem. Ebia nea enti a ɛte saa ne biribi a wontee ho asɛm da. Ɛnnɛ, yɛbɛka SAIDs (Systemic Autoinflammatory Diseases) a ɛyɛ den sɛ wɔbɛte ase na ebetumi ama obi anya atiridii mpɛn pii no ho asɛm. Bere bi a atwam no, na wɔfrɛ eyinom `(Periodic Fever Syndromes)` anaa `(Recurrent Fever Syndromes)`.

Dɛn ne saa SAID ahorow (Systemic Autoinflammatory Diseases) yi?

Sɛ yɛbɛka no tiawa a, SAID yɛ nyarewa kuw bi a ɛba esiane ɔhaw bi a yɛn nipadua no abɔde mu tumi a ɛko tia nyarewa no ntumi nyɛ adwuma yiye anaasɛ ɛhyɛ so nti . Nea ɛba ne sɛ, sɛ akyire biara nni hɔ a ɛde ba, kyerɛ sɛ, mmoawa anaa mmoawa bi nni hɔ a, nipadua no ma ɔfe ba kɛkɛ. Ɛno nti na atiridiinini a ɛtaa ba yi ba no.

Afei ebia woresusuw sɛ, "Oh, so eyi yɛ saa Autoimmune Diseases no mu biako?" Dabi, saa abien yi yɛ soronko kakra. Sɛ nhwɛso no, wɔ Autoimmune Diseases mu no, wɔ nyarewa te sɛ Rheumatoid Arthritis anaa Lupus mu no, yɛn nipadua no nipadua mu ahoɔden a ɛko tia nyarewa a ɛsakra no di mfomso tow hyɛ n’ankasa nkwammoaa a ɛte apɔw so. Nanso wɔ SAID ahorow mu no, ɔhaw no fi awosu mu nipadua mu ahoɔden a ɛko tia nyarewa no.

SAIDs ho yɛ na koraa sen Autoimmune Diseases. Mpɛn pii no, eyinom yɛ awosu mu nneɛma , a ɛkyerɛ sɛ awosu mu nsakrae anaa ɔkwan foforo bi na ɛde ba.

Saa SAID ahorow yi taa (nanso ɛnyɛ bere nyinaa) fi ase bere a wo ba yɛ akokoaa anaa abofra ketewaa . Abofra no benya yare no bere bi anaa ntua wɔ mmere bi mu. Wɔ saa nsɛm yi mu no, atiridiinini ne sɛnkyerɛnne afoforo betumi ada adi. Nanso, wɔ saa "ntua" yi ntam no, ebia abofra no nni sɛnkyerɛnne biara. Wɔnnya SAIDs ano aduru biara, nanso mpɛn pii no, ayaresa a etu mpɔn wɔ hɔ a wɔde siw sɛnkyerɛnne ahorow no ano.

Dɛn ne SAID ahorow titiriw?

Nhwehwɛmufo ahu SAID ahorow bɛyɛ 60, na wɔrehu pii. Ahorow a wɔtaa de di dwuma no bi ni:

• Abusua mu Mediterranea atiridii (FMF): Eyi ne nea abu so sen biara wɔ atiridiinini a ɛsan ba a efi awosu mu kyerɛ no mu. Ebetumi ama abofra no yafunu, ne koko, ne ne nkwaa ahonhon a ɛyɛ yaw.
• Atiridii a ɛba bere ne bere mu, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Eyi taa fi ase wɔ mmofra nkumaa mu, mpɛn pii no ansa na wɔadi mfe 4. Nanso, wɔ mmofra binom mu no, tebea yi betumi afi hɔ bere a wɔadi mfe 10 akyi.
• Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS): Eyi betumi aba fi mmofraase kosi mmerantebere ne mmabaabere mu, na ɛtɔ mmere bi a ɛkɔ mpanyin afe so.
• Mevalonate kinase deficiency (MKD): Kan no na wɔfrɛ no Hyper-IgD syndrome, na mpɛn pii no eyi 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’s disease (AOSD) a efi ase wɔ mpanyimfo mu: Sɛnea edin no kyerɛ no, saa yare yi fi ase wɔ mpanyin afe so. Ɛyɛ tebea bi a wɔfrɛ no systemic juvenile idiopathic arthritis (JIA) a ɛba mpanyimfo mu.
• NOD-2-associated granulomatous disease (Blau syndrome): Eyi nso 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 a ɛtaa ba ne atiridii a ɛba bere ne bere mu anaasɛ ɛba bere ne bere mu . Sɛnea yɛadi kan aka no, ebia abofra no ho bɛtɔ no wɔ mmere a atiridii nni mu no mu. Nanso, sɛnkyerɛnne afoforo wɔ hɔ a ɛfa SAID ahorow biara ho:

• Wɔ FMF mu: Abofra no yafunu, ne koko, ne ne nkwaa betumi ayɛ no yaw kɛse na ne ho ahonhon. Ɛtɔ da bi a, ebia honam ani akisikuru bɛba anan anaa anan ase.
• Wɔ PFAPA mu no: Menewa mu yaw, ano mu akuru, ntini a ɛho ahonhon wɔ kɔn mu. Eyinom ne sɛnkyerɛnne atitiriw.
• Wɔ TRAPS mu: Nipadua no betumi ayɛ awɔw ne atiridiinini. Ebia abofra no ntini mu yaw wɔ ne honam ani ne ne nsateaa mu. Akisikuru kɔkɔɔ a ɛyɛ yaw betumi apue, na ebetumi atrɛw afi abasa no so akɔ anan mu na afei akɔ nipadua no mu.
• Wɔ MKD mu: Wubetumi anya sɛnkyerɛnne ahorow a ɛte sɛ atiridii te sɛ awɔw, tiyɛ, yafunu mu yaw, aduan ho akɔnnɔ a ɛyera, ne sɛnkyerɛnne afoforo.
• Wɔ NLRP3 nyarewa mu no: honam ani akisikuru, tiyɛ, adwenemhaw, nkwaa mu yaw, ne aniwa a ɛyɛ hyew (conjunctivitis) betumi aba.
• Wɔ AOSD mu no: honam ani akisikuru, nkwaa mu yaw, ntini mu yaw. Nnipa binom nso betumi anya menewam yaw, yafunu mu yaw, ɔbrɛ, ne mmerɛwyɛ nka.
• Wɔ Blau syndrome mu: Akokoaa no betumi anya honam ani akuru wɔ ne nsa, ne nan, anaa ne mfinimfini. Ebia wɔn nkwaa mu yaw ne aniwa mu yaw nso.

Wo de susuw ho hwɛ, Nalini babea kumaa no anya atiridii kɛse nnansa kosi nnan ɔsram biara wɔ asram kakraa a atwam no mu. Ɛno ka ho no, ɔrenya akuru nketenkete nso wɔ n’anom ne akuru wɔ ne kɔn mu. Bere a ɔde kyerɛɛ oduruyɛfo bi no, ɔkae sɛ ebetumi ayɛ PFAPA.

Dɛn na ɛde SAID ahorow ba?

SAID dodow no ara yɛ awosu mu nyarewa . Eyi kyerɛ sɛ nsakrae a ɛba yɛn awosu mu nkwaadɔm mu na ɛde saa yare yi mu biara ba. SAID ahorow a ɛtaa ba ne awosu mu nkwaadɔm a ɛka ho ni:

• FMF: Awosu mu abɔde a wɔfrɛ no `(MEFV)`. Saa awosu mu abɔde yi kyerɛ sɛnea wɔyɛ protein `(pyrin)`.
• PFAPA: Wɔnnya nhuu awosu mu abɔde pɔtee a ɛka eyi.
• TRAPS: Awosu mu abɔde a wɔfrɛ no `(TNFRSF1A)`. Eyi ne baabi a akwankyerɛ a wɔde yɛ protein `(tumor necrosis factor receptor - TNFR)` no fi.
• MKD: Awosu mu abɔde a nkwa wom `(MVK)`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(mevalonic kinase)`.
• NLRP3 nyarewa: Awosu mu abɔde a wɔfrɛ no `(NLRP3)`. Eyi ma akwankyerɛ a wɔde bɛyɛ protein a wɔfrɛ no `(cryopyrin)`.
• AOSD: Wɔda so ara nnim nea enti pɔtee a ɛte saa.
• Blau yare: Awosu mu abɔde a nkwa wom `(NOD2)`. Eyi kyerɛ sɛnea wɔyɛ protein `(NOD2)`.

Ebia ɛbɛyɛ te sɛ nea awosu mu nkwaadɔm yi ho nsɛm a ɛkɔ akyiri no yɛ den kakra ma wo. Sɛ yɛbɛka no tiawa a, saa awosu mu nkwaadɔm yi nya protein ahorow bi a ɛhwɛ ɔfe so wɔ yɛn nipadua mu no so nkɛntɛnso. Ɛno nti na nipadua no ma ɔhyew tebea horow ba a ntease biara nnim no.

Sɛ wɔansa SAIDs no yiye a, dɛn na ebetumi asi?

Ɛho hia paa sɛ wonya ayaresa a ɛfata ma saa SAID ahorow yi. Efisɛ sɛ ɔfe a ɛwɔ nipadua no mu no kɔ so a, tebea bi a wɔfrɛ no `(amyloidosis)` betumi aba. Eyi kyerɛ sɛ protein bi si yɛn asaabo mu, na ebetumi asɛe asaabo no daa . Enti, sɛ yare no ho sɛnkyerɛnne bi wɔ hɔ a, ɛho hia paa sɛ wobɛhwehwɛ aduruyɛ mu afotu a wommu w’ani ngu so.

Ɔkwan bɛn so na nnuruyɛfo hu SAID ahorow?

SAID ahorow a wobehu no betumi ayɛ den efisɛ sɛnkyerɛnne ahorow no betumi ayɛ te sɛ tebea afoforo a emu yɛ den te sɛ lupus anaa lymphoma de. Enti , ɛbɛyɛ papa sɛ wobɛkɔ akɔhwɛ akisikuru ho oduruyɛfo, oduruyɛfo a wanya ntetee titiriw wɔ nyarewa a ɛma obi ho hyehye no ho , na wahu wo ba no tebea no pɛpɛɛpɛ na wahwɛ so.

Wo ba no ntini ho ɔyaresafo bɛhwɛ nneɛma pii de ahu SAIDs. Obebisa wo wo ba no yare no ho sɛnkyerɛnne ne sɛ ebia obi wɔ w’abusua mu a wanya atiridii mpɛn pii anaa. Oduruyɛfo no betumi asusuw sɛ tebea yi wɔ hɔ sɛ:

• Sɛ abofra no taa nya atiridii a .
• Sɛ obi wɔ abusua no mu a ɔwɔ abakɔsɛm a ɛkyerɛ sɛ ɔtaa nya atiridii a .
• Sɛ abofra no fi abusuakuw bi a ɛda adi sɛ ɛtaa nya atiridiinini a ɛte saa mu a.

Sɔhwɛ ahorow bɛn na wɔde di dwuma?

Nea ɛbɛyɛ na wo ba no akisikuru ho ɔbenfo betumi akamfo akyerɛ sɛ wɔnyɛ nhwehwɛmu pii. Eyinom bi ne:

• Lab nhwehwɛmu: Mogya mu nhwehwɛmu 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 wowɔ atiridiinini na wosan kɔ sɛnea na ɛte kan bere a atiridii no ano abrɛ ase no.
• Nsu mu nhwehwɛmu: Wɔhwɛ sɛ protein pii wɔ nsu mu anaa. Wɔ MKD ho no, wɔsan nso hwɛ nsuo no mu sɛ ebia organic acid a wɔfrɛ no ``mevalonic acid'' no dɔɔso anaa.
• Awosu mu nhwehwɛmu: Awosu mu nhwehwɛmu betumi ahwɛ sɛ awosu mu nneɛma ahorow a yɛaka ho asɛm wɔ atifi hɔ no wɔ hɔ anaa. Nanso, ebia mmofra binom a wɔwɔ SAID no nni awosu mu nsakrae ahorow a wɔahu no, enti ebia nea efi nhwehwɛmu no mu ba no nyɛ papa.

Dɛn ne ayaresa ahorow a wɔde sa SAIDs?

SAID ahorow no ayaresa gyina tebea no ko ne sɛnea emu yɛ den so . Biribiara nni hɔ a wɔde sa tebea horow yi, nanso nnuru betumi aboa ma woadi wo ba no yare no ho sɛnkyerɛnne ahorow so . Sɛ wo ba no nya ntua kakraa bi pɛ afe biara a, mpɛn pii no wubetumi de nnuru a wɔde kum ɛyaw ne nnuru a ɛnyɛ steroid a ɛko tia ɔyare mmoawa (NSAID) adi sɛnkyerɛnne ahorow no ho dwuma. Nanso, sɛ tebea no mu yɛ den kɛse a, wo duruyɛfo betumi akamfo ayaresa afoforo akyerɛ:

• FMF: Wobetumi de aduru bi a wɔfrɛ no `(Colchicine)` ayɛ eyi de atew ɔfe so. Sɛ wɔantumi amfa `(Colchicine)` amma abofra no a, wobɛtumi asɔ `(biologic)` aduru bi a wɔfrɛ no `(Canakinumab)` ahwɛ.
• PFAPA: Steroid te sɛ prednisone betumi atew bere tenten a PFAPA "ntua" di so. Cimetidine, aduru bi a wɔde ma mmofra binom ma yafunu mu akisikuru nso betumi aboa ma yare no ho sɛnkyerɛnne no afi hɔ.
• TRAPS: Aduru `(Canakinumab)` no yɛ adwuma yiye ma TRAPS. Afei nso, wobetumi de nnuru a ekum ɔyare mmoawa te sɛ `(glucocorticoids)` a oduruyɛfo no akyerɛw ama no adi sɛnkyerɛnne ahorow no so.
• MKD: `(Canakinumab)` nso yɛ aduru a etu mpɔn a wɔde sa MKD. `(NSAIDs)` anaa `(steroids)` betumi aboa wɔ "ntua" bere mu.
• NLRP3 nyarewa: Wɔde nnuru a ɛma nipadua no tumi ko tia nyarewa te sɛ Canakinumab, Rilonacept, ne Anakinra adi dwuma yiye de asa nyarewa a ɛfa NLRP3 ho.
• AOSD: Wɔde nnuru ahorow a ekum ɔyare mmoawa te sɛ steroids, nnuru a ɛko tia akisikuru a ɛsakra yare (DMARDs), ne nnuru a wɔde sa AOSD di dwuma de sa AOSD.
• 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.

Sɛ wokenkan nnuru yi ho asɛm a, ebia ɛbɛma woasuro kakra. Nanso kae sɛ, wɔde nnuru yi nyinaa ma wɔ oduruyɛfo hwɛ so denneennen, wɔ ɔkwan a ɛfata abofra no yiye so. Enti, ɛho hia sɛ wodi ayaresa no akyi pɛpɛɛpɛ sɛnea oduruyɛfo no ka no.

So SAIDs tebea no rekɔ?

SAID ahorow bi yɛ nkwa nna nyinaa tebea horow . Ebia afoforo nso bɛtra hɔ mfe kakraa bi na bere a wɔrenyin no akɔ . Tebea horow bi kɔ so wɔ nkwa nna nyinaa mu, nanso bere kɔ so no, mpɛn dodow a ntua ba no so betumi atew na yare no ho sɛnkyerɛnne ahorow no anyɛ den kɛse. Wo duruyɛfo bɛkyerɛkyerɛ wo ba no tebea pɔtee mu akyerɛ wo.

Abofra a ɔwɔ SAID a wɔbɛhwɛ no betumi ayɛ den. Sɛ w’ankasa woaka a, wubetumi de wo suahu no aboa wɔ wo ba no hwɛ mu. Wo ntease nso ho hia wɔ boa a wobɛboa wo ba ma watra ase ne saa nkwa nna nyinaa tebea yi mu.

Ade a ɛho hia sen biara ne sɛ wobɛhwehwɛ ayaresa afi nnuruyɛfo a wɔn ho akokwaw a wonim SAIDs ho nimdeɛ 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 yɛnsan nsusuw nsɛntitiriw a ɛsɛ sɛ wokae fi nea yɛaka ho asɛm no mu no bi ho:

• Sɛ wo ba no taa nya atiridiinini a wontumi nkyerɛkyerɛ mu a , ebetumi ayɛ SAID ho sɛnkyerɛnne.
• Ɛnyɛ ɔyare mmoawa na ɛde SAIDs ba , na mmom ɛyɛ ɔhaw a ɛwɔ nipadua no ankasa nipadua mu ahoɔden a ɛko tia nyarewa no ho.
• Eyinom yɛ nyarewa a wɔntaa nhu a mpɛn pii no awosu mu nneɛma na ɛde ba .
• Ebia ɛsono sɛnkyerɛnne ahorow a egyina SAID ko a ɛwɔ so no so, nanso atiridiinini a ɛtaa ba ne sɛnkyerɛnne titiriw no .
• Ɛho hia sɛ wokɔ Rheumatologist nkyɛn kɔhwehwɛ sɛ wohu yare no yiye na wɔsa no yare.
• Ɛwom sɛ ayaresa ntumi nsa yare no koraa de, nanso wobetumi adi sɛnkyerɛnne ahorow no so yiye .
• Mma w’ani ngu yare no ho sɛnkyerɛnne ahorow so, efisɛ sɛ wɔansa a , ebetumi ama asaabo asɛe .

Sɛ wowɔ nsɛmmisa foforo bi fa eyi ho a, ntwentwɛn wo nan ase sɛ wo ne w’abusua oduruyɛfo anaa oduruyɛfo a ɔyɛ akisikuru bɛkasa. Wɔbɛtumi aboa wo akɔ akyiri.

` SAIDs, atiridiinini a ɛba bere ne bere mu, atiridiinini a ɛsan ba, nyarewa a ɛma obi ho yɛ hyew, atiridiinini, atiridiinini wɔ mmofra mu, awosu mu nyarewa