Yala farigan bɛ to den na wa? Yala a bɛ se ka kɛ SAIDw (Systemic Autoinflammatory Diseases) sababu ye wa?

Oh, yala i denmisɛnnin bɛ to ka sumaya sɔrɔ nin donw in na wa? Ni i b’a miiri ko i kɛnɛyara tuma min na dɔrɔn, yala a bɛ wuli tuguni wa? Tuma dɔw la, o sababu tɛ se ka kɛ banakisɛ walima banakisɛ caman ye. Bi an bɛna kuma bana dɔ kan min ka gɛlɛn dɔɔnin, nka a nafa ka bon kosɛbɛ ka o dɔn. O bɛ wele ko SAIDs, o kɔrɔ ye ko Systemic Autoinflammatory Diseases . Fɔlɔ, o tun bɛ wele ko `(Sindromes périodiques)` walima `(Syndromes récurrents de fever)`.

SAIDw ye mun ye? An k’a faamu cogo nɔgɔman na.

N’an y’a fɔ cogo nɔgɔman na, SAIDw ye bana kulu ye minnu bɛ sɔrɔ an farikolo farikolo tangacogo bangenen baarakɛbaliya walima sariyako gɛlɛya fɛ . O de kama den bɛ se ka farigan sɔrɔ tuma caman hali ni banakisɛ tɛ a la (i n’a fɔ banakisɛ walima banakisɛ).

Min nafa ka bon kosɛbɛ, o ye ko nin bana minnu bɛ wele ko SAIDs, olu man kan ka ɲagami ni `(Autoimmune Diseases)` ye (misali la `(Rheumatoid Arthritis)` walima `(Lupus)` minnu bɛ se ka mɛn. `(Autoimmune)` banaw la, ka da fili dɔw kan an farikolo `(adaptive immune system)` la, o farikolo bɛ bin a yɛrɛ ka kɛnɛya selilɛriw kan. Nka SAIDw kɔnɔ, fɛn wɛrɛ bɛ kɛ.

SAIDw ka dɔgɔn kosɛbɛ ka tɛmɛ farikolo tangalanw ka banaw kan. a ka c' a la , u bɛ sɔrɔ cɛya la , wa u bɛ sɔrɔ jamu jiginni fɛ .

A ka ca a la, nka a tɛ kɛ tuma bɛɛ, SAID taamasiɲɛw bɛ daminɛ ni den ka dɔgɔ kosɛbɛ, a denmisɛnman walima a denmisɛnman . Sumaya ni taamasiɲɛ wɛrɛw bɛ se ka kɛ aw den na ni nin bana in ye. Nin waati ninnu ni ɲɔgɔn cɛ, a bɛ se ka kɛ ko taamasiɲɛ foyi tɛ aw den na fewu. Hali ni fura tɛ SAIDw na, a ka ca a la furakɛli dɔw bɛ yen minnu bɛ aw dɛmɛ ka aw den ka bana taamasiɲɛw kunbɛn.

SAID suguya jɔnjɔnw ye jumɛnw ye?

Ɲininikalaw ye SAID suguya 60 ɲɔgɔn Sɔrɔ, wa kura bɛ ka Sɔrɔ hali bì. SAID suguya minnu ka ca kosɛbɛ, olu dɔw filɛ nin ye:

• Denbaya ka Mediterane sumaya (FMF): Nin ye sumayabana ye min ka teli ka sɔrɔ jamu fɛ, sumaya min bɛ segin ka kɛ. A bɛ se ka kɛ sababu ye ka denmisɛnniw kɔnɔbara, u disi ani u kolotugudaw funu ni dimi ye.
• Sumaya waati ni waati, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): A ka ca a la, o bɛ daminɛ denmisɛnni fitiniw na sani u ka se san 4. Tuma dɔw la, bana bɛ se ka kɛnɛya a yɛrɛ ma san 10 kɔfɛ.
• Tumu nécrose facteur receptor-associé périodique syndrome (TRAPS): O bɛ se ka kɛ denmisɛnya la, funankɛya la, walima hali balikuya la.
• Mevalonate kinase dɛsɛ (MKD): A tun bɛ wele fɔlɔ ko ``Hyper-IgD syndrome'', a ka ca a la o bɛ daminɛ sani den ka san kelen sɔrɔ.
• NLRP3 ni farikolojidɛsɛbana minnu bɛ sɔrɔ:O tun bɛ wele fɔlɔ ko `(Cryopyrin-associés périodiques syndromes - CAPS)`. Sugu fitinin saba wɛrɛw bɛ o kɔnɔ.
• Bana min bɛ daminɛ balikuw fɛ (AOSD): I n’a fɔ a tɔgɔ b’a jira cogo min na, nin ye denmisɛnniw ka bana ye min bɛ daminɛ balikuw fɛ ``Systemic Juvenile Idiopathic Arthritis (JIA)``.
• NOD-2-associé granulomatose disease (Blau syndrome): A ka ca a la, o bɛ daminɛ sani a si san 4. A bɛ den fari, a ɲɛw ani a kolotugudaw de minɛ kosɛbɛ.

SAIDw taamasiɲɛw ye mun ye?

A ka ca a la, SAID taamasiɲɛw bɛ daminɛ denmisɛnya la. a taamasiɲɛba ni a ka ca a la , o ye sumaya ye min bɛ kɛ tuma ni tuma . Sumaya waatiw la, a ka ca a la, mɔgɔw bɛ se ka kɛnɛya, a taamasiɲɛ foyi tɛ u la. Nka, taamasiɲɛ wɛrɛw bɛ se ka sɔrɔ minnu kɛrɛnkɛrɛnnen don SAID suguya kelen-kelen bɛɛ la. An k’a lajɛ u ye mun ye:

• FMF : O bɛ se ka funu ni dimi juguman bila den kɔnɔbara la, a disi la ani a kolotugudaw la . Kurukuruni bɛ se ka kɛ senkɔniw duguma walima senkɔniw fana na.
• PFAPA : O bɛ se ka kɛ sababu ye ka galaka dimi, da kɔnɔdimi, ani ka ɲɛgɛnɛsira funu kɔ la . Misali la, ni aw den bɛ taamasiɲɛw jira i n’a fɔ tonso ka to ka kirin ani da kɔnɔdimi, o bɛ se ka kɛ PFAPA ye.
• JANW: O bɛ se ka farigan ni farikolodimi bila senkɔni/kɔnɔna ni bolokɔniw na . A bɛ se ka kuru bilenman dimi fana bila mɔgɔ la min bɛ daminɛ bolokɔniw ni senw na ka jɛnsɛn farikolo la.
• MKD: O bɛ se ka kɛ sababu ye ka bana taamasiɲɛw bila mɔgɔ la i n’a fɔ farigan, kungolodimi, kɔnɔdimi, dumuni nege tɛ mɔgɔ la, ani bana minnu bɛ i n’a fɔ mura .
• NLRP3 banaw : Nin bana in bɛ se ka farikolojidɛsɛ bila mɔgɔ la, kungolodimi, farigan, kolotugudaw dimi, ani ɲɛ/ɲɛdimi pinki .
• AOSD: Nin ye bana ye min ka teli ka kɛ sababu ye ka fari kurukuruw, kolotugudaw dimi, ani farikolodimi bila mɔgɔ la . Mɔgɔ dɔw fana bɛ se ka dadimi, kɔnɔdimi, sɛgɛn ani farigan.
• Blau syndrome : O bɛ se ka fari joginda bila den bolow la, a senw na walima a cɛmancɛ yɔrɔ la . a bɛ se ka kolodimi ni ɲɛdimi fana bila mɔgɔ la .

SAIDw sababu ye mun ye?

SAID caman ye banaw ye minnu bɛ sɔrɔ jamu fɛ . O kɔrɔ ye ko fɛn dɔ jiginni kɛrɛnkɛrɛnnen (variant) min bɛ sɔrɔ jɛnɛya kɛrɛnkɛrɛnnen dɔ la, o bɛ kɛ sababu ye ka sindimɛ kelen-kelen bɛɛ lase mɔgɔ ma. Miiri k’a filɛ, cikanw (jeniw) dɔw b’an farikolo la minnu b’a fɔ an ye baara kelen-kelen bɛɛ kɛcogo, o la ni o gafe kɔnɔ sɛbɛnni kelen ma ɲɛ, o bɛ se ka kɛ sababu ye ka baara tɔ kɛ ka ɲɛ.

SAID suguya kunbabaw ni jamu minnu bɛ tali kɛ u la, olu filɛ nin ye:

• FMF: 1 .Jɛnɛya `MEFV` ye min bɛ cikanw di farikolojɔlifɛn `(pirini)` dilancogo kan.
• PFAPA: Nin sababu tigitigi ma dɔn fɔlɔ .
• TRAPS: Jɛnkulu ye `TNFRSF1A` ye. O bɛ cikan di farikolojɔlifɛn dɔ dilanni ma min bɛ wele ko `(tumor necrosis factor receptor - TNFR)`.
• MDK: Jɛnkulu min bɛ wele ko `MVK`. O bɛ cikan di farikolojɔlifɛn dɔ dilanni ma min bɛ wele ko `(mevalonic kinase)`.
• NLRP3 banaw: Jɛnɛya `NLRP3`. O bɛ cikan di farikolojɔlifɛn dɔ dilanni ma min bɛ wele ko `(cryopyrin)`.
• AOSD: O sababu tigitigi ma sɔrɔ fɔlɔ .
• Blau ka bana: O ye jɛnɛya ye min bɛ wele ko `NOD2`. O bɛ cikan di farikolojɔlifɛn dɔ dilanni ma min bɛ wele ko `(NOD2)`.

A nafa ka bon k’a faamu ko ni ninnu sababu bɔra jamu fɛnw na, o tɛ bangebagaw ka jalaki ye. Olu ye ɲɛnamaya kɛcogo ye min ka gɛlɛn kosɛbɛ.

Gɛlɛya minnu bɛ sɔrɔ SAIDw fɛ

A nafa ka bon kosɛbɛ ka furakɛli ɲuman sɔrɔ o bana ninnu na. Sabula, ni funu tora fari la, o bɛ se ka kɛ sababu ye ka bana dɔ lase mɔgɔ ma min bɛ wele ko `(Amyloidose). `(Amyloidose)` ye farikolojɔli dumuni ye min bɛ sɔrɔ sugunɛ na . o bɛ se ka sugunɛ tiɲɛ kudayi . O la, ni bana taamasiɲɛw bɛ yen, a nafa ka bon ka dɔgɔtɔrɔso laadilikan ɲini joona.

SAIDw bɛ dɔn cogo di?

Ni an bɛ se ka tiɲɛ fɔ, SAIDw sɛgɛsɛgɛli bɛ se ka gɛlɛya dɔɔni barisa a taamasiɲɛw bɛ se ka kɛ i n’a fɔ bana jugu wɛrɛw, i n’a fɔ lupus walima lymphome. O la, a nafa ka bon ka taa dɔgɔtɔrɔ dɔ fɛ min ka kalan kɛrɛnkɛrɛnnen bɛ funubanaw la (sɔgɔsɔgɔninjɛ furakɛla) walasa ka den ka bana sɛgɛsɛgɛ ka ɲɛ ani k’a ɲɛnabɔ.

Aw den ka sɔgɔsɔgɔninjɛ dɔgɔtɔrɔ bɛna baara kɛ ni fɛn caman ye walasa ka SAIDw sɛgɛsɛgɛ. A bɛna ɲininkali kɛ aw den ka bana taamasiɲɛw la ani ni aw ka denbaya kɔnɔ mɔgɔ dɔ ye nin bana ninnu sɔrɔ (denbaya ka tariku biologique). Dɔgɔtɔrɔ bɛ se ka sigasiga ko sumayabana basigilen bɛ aw den na ni:

• Ni farigan bɛ to aw la
• Ni mɔgɔ o mɔgɔ bɛ denbaya kɔnɔ, ni farigan bɛ a la waati ni waati i n’a fɔ nin
• Ni aw bɛ mɔgɔ kulu dɔ la minnu ka teli ka o sumaya suguw sɔrɔ

Kɔrɔbɔli jumɛnw bɛ kɛ?

Aw ka dɔgɔtɔrɔ bɛ se ka sɛgɛsɛgɛli caman kɛ walasa ka bana in sɛgɛsɛgɛ. Olu bɛ se ka kɛ ninnu ye:

• Laboratowari sɛgɛsɛgɛliw : Sɛgɛsɛgɛliw i n’a fɔ `(C-reactive protein - CRP)` ani `(Complete Blood Count - CBC)` bɛ se ka funu lajɛ farikolo la. Nin sɛgɛsɛgɛli ninnu bɛ kɛ `(positif)` ye ni sumaya bɛ a la, ka segin u cogo kɔrɔ la ni sumaya banna.
• Sugunɛ sɛgɛsɛgɛli:Sugunɛ furakisɛ sɛgɛsɛgɛli bɛ se ka kɛ walasa ka a dɔn ni farikolojɔli dumuni caman bɛ sugunɛ na. MKD la, sugunɛ sɛgɛsɛgɛli min kɛra asidi fɛnɲɛnamaw kan, o bɛna asidi mevaloniki hakɛ caman jira.
• Jɛnkulu sɛgɛsɛgɛli: O bɛ se ka jamu suguya wɛrɛw lajɛ. Nka, SAID bɛ denmisɛn dɔw la, a bɛ se ka kɛ ko o jamu suguya tɛ u la. O la, hali ni sɛgɛsɛgɛli jaabiw ye ``negatif`` ye, SAID sɔrɔli tɛ se ka bɔ kɛnɛ kan.

SAIDw bɛ furakɛ cogo di?

SAIDw furakɛli bɛ danfara ka kɛɲɛ ni bana suguya ni a juguya ye . Hali ni nin bana ninnu tɛ se ka furakɛ pewu, den taamasiɲɛw bɛ se ka kunbɛn ka ɲɛ ni furaw tali ye . Misali la, ni aw den bɛ nin farigan ninnu sɔrɔ siɲɛ damadɔ dɔrɔn san kɔnɔ, a ka ca a la dimimadafuraw ni dimimadafura minnu tɛ stɛroyi ye (AINE) olu bɛ se ka a taamasiɲɛw kunbɛn. Nka, ni bana juguyara ka tɛmɛ, dɔgɔtɔrɔ bɛ se ka furakɛcogo wɛrɛw fɔ.

O furakɛli sugu dɔw filɛ nin ye:

• FMF : O bɛ se ka furakɛ ni fura ye min bɛ wele ko `(Colchicine)` walasa ka funu dɔgɔya. Ni aw tɛ se ka `(Colchicine)` ta, aw bɛ se ka fura suguya `(Biologic)` dɔ kɛ k'a lajɛ min bɛ wele ko `(Canakinumab)`.
• PFAPA : PFAPA ka wulicogo bɛ se ka surunya ni furakisɛw (a ka ca a la piridinizɔni) dili ye. Denmisɛn dɔw sera k’u ka bana taamasiɲɛw kunbɛn ni simetidini tali ye, o ye fura ye min bɛ kɛ ka kɔnɔboli furakɛ.
• TRAPS: Fura `(Canakinumab)` bɛ se ka kɛ kosɛbɛ TRAPS la. Ani fana, fura minnu bɛ sɔrɔ dɔgɔtɔrɔso la i n’a fɔ `(Glucocorticoïdes)`, olu fana bɛ se ka bana taamasiɲɛw nɔgɔya.
• MKD: `(Canakinumab)` ye fɛɛrɛ ɲuman ye MKD furakɛli la. Ni aw bɛ `(ANS)` walima `(Stéroïdes)` kɛ farigan waati la, o fana bɛ se ka kɛ dɛmɛ ye.
• NLRP3 banaw : ``Immunomodulators'' fura wɛrɛw i n'a fɔ ``Canakinumab'' ani ``Rilonacept'' ani ``Anakinra'' bɛ kɛ ka NLRP3 banaw furakɛ ka ɲɛ.
• AOSD : Fura suguya caman bɛ kɛ ka AOSD furakɛ. Olu ye `(Stéroïdes)`, `(Bana caman sɛmɛntiya furaw - DMARDs)`, ani `(Biologics)`.
• Blau ka bana : Ka kɛɲɛ ni den ka bana taamasiɲɛw ye, farikolo tangalanw, tumor necrosis factor (TNF) inhibiteurs, ani/walima ɲɛfura minnu bɛ kɛ fari la, olu bɛ se ka kɛ.

Yala SAIDw cogoya bɛ ɲɛ a yɛrɛ ma wa?

SAID dɔw bɛ se ka mɛn u si bɛɛ la . Dɔ wɛrɛw bɛ se ka mɛn san damadɔ kɔnɔ ani u bɛ se ka kɛnɛya ni u kɔrɔbayara. Hali ni bana dɔw bɛ kɛ mɔgɔ si bɛɛ la, sumaya hakɛ bɛ se ka dɔgɔya waati tɛmɛnen kɔfɛ ani a taamasiɲɛw bɛ se ka juguya kosɛbɛ . Aw ka dɔgɔtɔrɔ bɛ se ka kunnafoni kɛrɛnkɛrɛnnenw di aw ma aw den ka bana kɛrɛnkɛrɛnnen kan.

SAID bɛ den min na, o ladonni bɛ se ka kɛ gɛlɛya ye. Laala aw yɛrɛ ye SAID kɛ, nka aw bɛ se ka baara kɛ ni aw yɛrɛ ka ko kɛlenw ye walasa ka aw den ladonni dɛmɛ ani k’u dɛmɛ u ka se ka nin bana in kunbɛn a si bɛɛ kɔnɔ.

Min nafa ka bon kosɛbɛ, o ye ka furakɛli ɲini dɔgɔtɔrɔ kɛrɛnkɛrɛnnenw fɛ minnu bɛ SAIDw ka gɛlɛyaw dɔn kosɛbɛ. U bɛ se ka dɛmɛ don ka den ka bana taamasiɲɛw kunbɛn ani ka a dɛmɛ ka denmisɛnya ɲuman sɔrɔ.

Fɛn minnu nafa ka bon kosɛbɛ an ka kan ka to an hakili la (Take-Home Message) .

O bɛ ɲɛ, o la, an ka kan ka fɛn dɔw lajɛ minnu ka kan ka to i hakili la ka bɔ an ye kuma minnu kan.

• Ni sumaya bɛ to den na, min ɲɛfɔlen tɛ , aw bɛ kuma dɔgɔtɔrɔ dɔ fɛ o ko la. A bɛ se ka kɛ SAID taamasyɛn ye.
• SAIDw ye farikolo tangacogo bangenen gɛlɛya ye , a tɛ banakisɛ ye min bɛ sɔrɔ tuma caman.
• ninnu ni ``Autoimmune'' banaw tɛ kelen ye .
• Tuma caman na, o fɛnw bɛ sɔrɔ jamu fɛnw fɛ .
• A nafa ka bon ka taa sɔgɔsɔgɔninjɛ furakɛla dɔ fɛ walasa ka bana sɛgɛsɛgɛli tigitigi sɔrɔ.
• furakɛli bɛ se ka bana taamasiɲɛw kunbɛn ka ɲɛ .
• Furakɛli joona nafa ka bon walasa ka sugunɛ tiɲɛni bali.

Ni ɲininkali wɛrɛw bɛ aw fɛ o ko la, aw kana siran ka aw ka denbaya dɔgɔtɔrɔ walima denmisɛnw ka dɔgɔtɔrɔ ɲininka. U bɛna se k’i dɛmɛ ka taa a fɛ.

` SAIDw, sumaya ciyɛntalen, sumaya ka teli, sumaya denmisɛnw na, farikolo tangacogo, jamu banaw, sɔgɔsɔgɔninjɛ furakɛla