Yala sumaya bɛ kɛ den na tuma caman na min ɲɛfɔlen tɛ wa? O bɛ se ka kɛ sababu ye ka SAIDw (Systemic Autoinflammatory Diseases) sɔrɔ!

Yala sumaya bɛ i denmisɛnnin na tuma bɛɛ wa? Tuma dɔw la, farigan bɛ u minɛ siɲɛ kelen walima siɲɛ fila kalo kɔnɔ, nka dɔgɔtɔrɔw b’a fɔ ko banakisɛ si tɛ farikolo la, o kɔrɔ ye ko banakisɛ walima banakisɛ si tɛ farikolo la. O waati suguw la, i kɛlen ba walima fa, i bɛ se kosɛbɛ ka siran ni jɔrɔnanko caman sɔrɔ. "Mun na nin bɛ ka kɛ ne den na?" N’a sɔrɔ i ye miiri siɲɛ ba kelen. Laala o kun ye fɛn ye i ma deli ka min mɛn fɔlɔ. Bi, an bɛna kuma SAIDw (Systemic Autoinflammatory Diseases) kan, minnu faamuyali ka gɛlɛn, wa u bɛ se ka farigan bila mɔgɔ la tuma caman. Fɔlɔ, olu tun bɛ wele ko `(Sindromes périodiques)` walima `(Syndromes récurrents de fever)`.

Nin SAID (bana minnu bɛ sɔrɔ farikolo la, olu ye mun ye)?

N’an y’a fɔ cogo nɔgɔman na, SAIDw ye bana kulu ye minnu bɛ sɔrɔ ka a sababu kɛ baarakɛbaliya walima a kunbɛnni gɛlɛya ye an farikolo farikolo tangacogo la . Min bɛ kɛ, o ye ko, ni kɛnɛma sababu si tɛ, o kɔrɔ ye ko banakisɛ walima banakisɛ, farikolo bɛ funu dɔrɔn de lawuli. O de y’a to nin farigan min bɛ to ka na.

Sisan, i bɛ se ka miiri ko: “O, yala nin ye o Autoimmune Diseases dɔ ye wa?” Ayi, nin fila in tɛ kelen ye dɔɔnin. Misali la, farikolo tangalanw na, banaw la i n’a fɔ sɔgɔsɔgɔninjɛ walima Lupus, an farikolo farikolo tangalan min bɛ se ka ladamu, o bɛ fili ka bin a yɛrɛ ka farikolokisɛ kɛnɛmanw kan. Nka SAIDw la, gɛlɛya bɛ farikolo tangacogo bangenen de la.

SAIDw ka dɔgɔ kosɛbɛ ka tɛmɛ farikolo tangalanw kan. A ka c’a la, ninnu ye ciyɛn ye , o kɔrɔ ye k’u bɛ sɔrɔ jamu jiginni walima fɛn wɛrɛ fɛ.

A ka ca a la (nka a tɛ kɛ tuma bɛɛ) nin SAID ninnu bɛ daminɛ ni aw den ye denmisɛn ye walima ni denmisɛnnin ye . Den bɛna bana in kɛ waati dɔw la walima a bɛna a minɛ waati dɔw la. Nin waati ninnu na, farigan ni taamasiɲɛ wɛrɛw bɛ se ka ye. Nka, nin "binkanni" ninnu cɛ, a bɛ se ka kɛ ko taamasiɲɛ tɛ den na. Fura tɛ SAIDw la, nka a ka ca a la furakɛli nafamaw bɛ yen walasa ka bana taamasiɲɛw kunbɛn.

SAID suguya jɔnjɔnw ye jumɛnw ye?

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

• Denbaya ka Mediterane sumaya (FMF): Nin ye sumayabana ye min ka teli ka sɔrɔ sumayabanaw la minnu bɛ sɔrɔ jamu fɛ, minnu bɛ segin-ka-bɔ. A bɛ se ka funu dimima don den kɔnɔbara la, a disi la ani a kolotugudaw la.
• Sumaya waati ni waati, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): A ka ca a la, o bɛ daminɛ denmisɛnni fitiniw na, a ka ca a la sani u ka se san 4. Nka, denmisɛn dɔw la, o bana bɛ se ka ban u si san 10 kɔfɛ.
• Tumu nekɔrɔsi facteur receptor-associé périodique fever syndrome (TRAPS): O bɛ se ka kɛ kabini denmisɛnya fo ka se balikuya ma, ani tuma dɔw la fo ka se balikuya ma.
• 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-associé autoinflammatoires banaw : O tun bɛ wele fɔlɔ ko Cryopyrin-associé périodique 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, o bana bɛ daminɛ balikuya la. O ye balikukalan suguya ye min bɛ wele ko systémique juvenile idiopathique arthritis (JIA).
• NOD-2-associé granulomatose disease (Blau syndrome): A ka ca a la o fana bɛ daminɛ sani a ka 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 denmisɛnni daminɛ na. a taamasiɲɛba ni a ka ca a la , o ye sumaya ye min bɛ kɛ tuma ni tuma walima waati ni waati . I n’a fɔ a fɔra cogo min ka tɛmɛ, den bɛ se ka kɛnɛya waati minnu na farigan tɛ a la. Nka, taamasiɲɛ wɛrɛw bɛ yen minnu bɛ kɛrɛnkɛrɛnnenya la SAID suguya kelen-kelen bɛɛ la:

• FMF kɔnɔ : Den kɔnɔbara, a disi ani a kolotugudaw bɛ se ka dimi kosɛbɛ ani ka funu. Tuma dɔw la, farilaji bɛ se ka kɛ senkɔniw duguma walima senkɔniw na.
• PFAPA la: Galaka dimi, da kɔnɔ ŋɛɲɛ, ɲɛgɛnɛsira funu kɔkolo la. Olu de ye a taamasiɲɛ kunbabaw ye.
• TRAPS kɔnɔ: Farikolo bɛ se ka farigan ni farigan ye. Farikolodimi bɛ se ka kɛ den fari la ani a bolokɔniw na. Kuru bilenman dimi bɛ se ka bɔ, o bɛ se ka bɔ bolokɔniw na ka taa senw na, ka tila ka taa farikolo la.
• MKD la: Aw bɛ se ka mura taamasiɲɛw sɔrɔ i n’a fɔ farigan, kungolodimi, kɔnɔdimi, dumuni nege tɛ aw la, ani taamasiɲɛ wɛrɛw.
• NLRP3 banaw la: fariŋɛɲɛ, kungolodimi, farigan, kolotugudaw dimi, ani ɲɛ funu (ɲɛdimi) bɛ se ka kɛ.
• AOSD la: fariŋɛɲɛ, kolotugudaw dimi, farikolodimi. Mɔgɔ dɔw fana bɛ se ka galodimi, kɔnɔdimi, sɛgɛn, ani fanga dɔgɔyali ye.
• Blau ka bana la: Den bɛ se ka fari joginda sɔrɔ a bolow, a senw walima a cɛmancɛ la. U bɛ se ka kolodimi ni ɲɛdimi fana sɔrɔ.

Miiri k’a filɛ, Nalini denmuso fitinin bɛ fariganba la kabini tile saba fo tile naani kalo o kalo kabini kalo damadɔ tɛmɛnenw. O ni ɲɔgɔn cɛ, joginda misɛnninw fana bɛ a da la ani kurukuruw bɛ a kɔ la. A y’a jira dɔgɔtɔrɔ dɔ la tuma min na, a ko a bɛ se ka kɛ PFAPA ye.

SAIDw sababu ye mun ye?

SAID fanba ye banaw ye minnu bɛ sɔrɔ jamu fɛ . O kɔrɔ ye ko nin bana ninnu kelen-kelen bɛɛ bɛ sɔrɔ an ka jamu caman ɲɔgɔnna yeli fɛ. SAID suguya minnu ka ca kosɛbɛ ani jamu minnu bɛ u nɔ bila, olu filɛ nin ye:

• FMF: Jɛnkulu min bɛ wele ko `(MEFV)`. O jɛnɛya in bɛ cikan di farikolojɔlifɛn `(pirini)` dilanni ma.
• PFAPA : Jɛnkulu tigitigi min bɛ nin ko in nɔ bila, o ma dɔn fɔlɔ.
• TRAPS: Jɛnkulu min bɛ wele ko `(TNFRSF1A)`. Nin yɔrɔ in de la, cikanw bɛ bɔ farikolojɔlifɛn `(tumor necrosis factor receptor - TNFR)` dilanni na.
• MKD: Jɛnkulu `(MVK)`. O bɛ cikan di farikolojɔlifɛn dɔ dilanni ma min bɛ wele ko `(mevalonic kinase)`.
• NLRP3 banaw : Jɛnɛya min bɛ wele ko `(NLRP3)`. O bɛ cikanw di farikolojɔlifɛn dɔ dilanni na min bɛ wele ko `(cryopyrin)`.
• AOSD: O kun tigitigi ma dɔn hali bi.
• Blau ka bana: Jɛnkulu `(NOD2)`. O bɛ cikan di farikolojɔlifɛn `(NOD2)` dilanni ma.

O jamu ninnu kunnafoni kunbabaw bɛ se ka kɛ i n’a fɔ u ka gɛlɛn dɔɔnin i bolo. N’an y’a fɔ cogo nɔgɔman na, o jamu ninnu bɛ nɔ bila farikolojɔlifɛn dɔw dilanni na minnu bɛ funu kunbɛn an farikolo la. O de kama farikolo bɛ funubanaw lase mɔgɔ ma kun tɛ.

Mun bɛ se ka kɛ ni SAIDw ma furakɛ ka ɲɛ?

A nafa ka bon kosɛbɛ ka furakɛli ɲuman sɔrɔ nin SAID ninnu na. Sabula ni funu bɛ to senna farikolo la, bana dɔ bɛ se ka sɔrɔ min bɛ wele ko `(amiloyidose)`. O kɔrɔ ye ko farikolojɔli dumuni suguya dɔ bɛ bila an ka sugunɛ na, o bɛ se ka sugunɛ tiɲɛ kudayi . O la, ni a taamasiɲɛw bɛ yen, a nafa ka bon kosɛbɛ ka dɔgɔtɔrɔso laadilikan ɲini ka sɔrɔ i ma i ɲɛmajɔ u la.

Dɔgɔtɔrɔw bɛ SAIDw sɛgɛsɛgɛ cogo di?

SAIDw sɛgɛsɛgɛli bɛ se ka kɛ gɛlɛya ye barisa a taamasiɲɛw bɛ se ka kɛ i n’a fɔ bana juguman wɛrɛw, i n’a fɔ lupus walima lymphome. O la , a ka fisa ka taa sɔgɔsɔgɔninjɛ dɔgɔtɔrɔ dɔ fɛ, dɔgɔtɔrɔ min ka kalan kɛrɛnkɛrɛnnen bɛ funubanaw la , walasa ka den ka bana sɛgɛsɛgɛ ka ɲɛ ani k’a ɲɛnabɔ.

Aw den ka sɔgɔsɔgɔninjɛ dɔgɔtɔrɔ bɛna fɛn caman lajɛ walasa ka SAIDw sɛgɛsɛgɛ. A bɛna aw ɲininka aw den ka bana taamasiɲɛw la ani ni mɔgɔ dɔ bɛ aw ka denbaya kɔnɔ min ye farigan sɔrɔ tuma caman. Dɔgɔtɔrɔ bɛ se ka sigasiga nin bana in na ni:

• Ni farigan bɛ to den na .
• ni denbaya kɔnɔ mɔgɔ o mɔgɔ ye sumaya sɔrɔ tuma caman na .
• Ni den ye siya dɔ ye min ka teli ka o sumaya suguw sɔrɔ tuma caman.

Kɔrɔbɔli jumɛnw bɛ kɛ?

Walasa ka bana sɛgɛsɛgɛli dafa, aw den ka sɔgɔsɔgɔninjɛ dɔgɔtɔrɔ bɛ se ka sɛgɛsɛgɛli caman kɛ. Olu dɔw ye ninnu ye:

• Lab sɛgɛsɛgɛliw : Joli 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 farigan bɛ aw la ani ka segin a cogo kɔrɔ la ni sumaya nɔgɔyara.
• Sugunɛ sɛgɛsɛgɛli : Sugunɛ bɛ lajɛ ni farikolojɔli dumuniw ka ca. MKD banaw la, sugunɛ fana bɛ lajɛ ni asidi organique dɔ hakɛ ka ca a la min bɛ wele ko ``asidi mevalonic''.
• Jɛnkulu sɛgɛsɛgɛli: Jɛnɛya sɛgɛsɛgɛli bɛ se ka jamu suguya minnu kofɔ sanfɛ, olu lajɛ. Nka, SAID bɛ denmisɛn dɔw la, a bɛ se ka kɛ ko jamu suguya minnu sɔrɔla, olu tɛ olu la, o la, sɛgɛsɛgɛli jaabiw bɛ se ka kɛ ko jugu ye.

SAIDw furakɛcogo jumɛnw bɛ yen?

SAIDw furakɛli bɛ bɔ bana suguya n' a juguya la . Fura tɛ nin bana ninnu na, nka furaw bɛ se ka dɛmɛ don ka den ka bana taamasiɲɛw kunbɛn . Ni bana dɔɔni dɔrɔn de bɛ den na san kɔnɔ, a ka ca a la, aw bɛ se ka a taamasiɲɛw kunbɛn ni dimimadafura ye ani ni dimimadafura minnu tɛ stɛroyi ye (AINE). Nka, ni bana in juguyara ka tɛmɛ, dɔgɔtɔrɔ bɛ se ka furakɛli wɛrɛw fɔ:

• FMF : O bɛ se ka furakɛ ni fura ye min bɛ wele ko `(Colchicine)` walasa ka funu dɔgɔya. Ni `(Colchicine)` tɛ se ka di den ma, aw bɛ se ka fura suguya `(biologic)` dɔ kɛ k'a lajɛ min bɛ wele ko `(Canakinumab)`.
• PFAPA : Stɛroyidiw i n’a fɔ piridinizɔni, olu bɛ se ka PFAPA “binkanni” kuntaala surunya. Simetidini, o ye fura ye min bɛ di denmisɛn dɔw ma kɔnɔboli kama, o fana bɛ se ka dɛmɛ don ka bana taamasiɲɛw lajɔ.
• TRAPS: Fura `(Canakinumab)` bɛ se ka kɛ kosɛbɛ TRAPS la. Ani fana, a taamasiɲɛw bɛ se ka kunbɛn ni banakisɛfagalanw ye i n’a fɔ `(glucocorticoïdes)` minnu sɛbɛnna dɔgɔtɔrɔ fɛ.
• MKD: `(Canakinumab)` fana ye MKD furakɛcogo ɲuman ye. `(ANS)` walima `(steroids)` bɛ se ka dɛmɛ don "binkanni" waati.
• NLRP3 banaw : Banakisɛfagalan minnu bɛ farikolo tangalanw i n’a fɔ Kanakinumab, Rilonacept ani Anakinra, olu kɛra ka ɲɛ ka banaw furakɛ minnu bɛ sɔrɔ NLRP3 fɛ.
• AOSD : Fura suguya caman bɛ kɛ ka AOSD furakɛ i n’a fɔ stɛroyi, bana caman sɛgɛsɛgɛli 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.

Ni aw ye fura ninnu kalan, o bɛ se ka siran dɔɔni bila aw la. Nka aw hakili to a la ko nin fura ninnu bɛɛ bɛ di dɔgɔtɔrɔ dɔ ka kɔlɔsiliba kɔnɔ, cogo la min bɛ bɛn den ma kosɛbɛ. O la, a nafa ka bon ka tugu furakɛli la tigitigi i n’a fɔ dɔgɔtɔrɔ y’a fɔ cogo min na.

Yala SAIDs cogoya bɛ ka ban wa?

SAID dɔw ye ɲɛnamaya bɛɛ kɔnɔko ye . dɔw bɛ se ka mɛn san damadɔ kɔnɔ ka ban ni u kɔrɔbayara . Bana dɔw bɛ kɛ mɔgɔ si bɛɛ la, nka waati tɛmɛnen kɔfɛ, binkanniw kɛcogo bɛ se ka dɔgɔya ani a taamasiɲɛw bɛ se ka juguya dɔɔni. Aw ka dɔgɔtɔrɔ bɛna aw den ka bana kɛrɛnkɛrɛnnenw ɲɛfɔ aw ye.

SAID bɛ den min na, o ladonni bɛ se ka kɛ gɛlɛya ye. Ni aw yɛrɛ ye SAID kɛ, aw bɛ se ka baara kɛ ni aw ka ko kɛlenw ye walasa ka dɛmɛ don aw den ladonni na. Aw ka faamuyali fana nafa ka bon walasa ka aw den dɛmɛ a ka ɲɛnamaya ni nin bana in ye a si bɛɛ kɔnɔ.

Min nafa ka bon kosɛbɛ, o ye ka furakɛli ɲini dɔgɔtɔrɔ ŋanaw fɛ minnu bɛ SAIDw dɔn. 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 sa, an ka segin ka hakilina jɔnjɔn dɔw lajɛ i ka kan ka minnu to i hakili la an ye kuma minnu kan:

• Ni sumaya bɛ to den na, min ɲɛfɔlen tɛ , o bɛ se ka kɛ SAID taamasiɲɛ ye.
• SAIDw tɛ sɔrɔ banakisɛ fɛ , nka u bɛ kɛ gɛlɛya ye farikolo yɛrɛ ka farikolo tangacogo la.
• ninnu ye banaw ye minnu man ca , minnu ka teli ka sɔrɔ jamu sababuw fɛ .
• A taamasiɲɛw bɛ se ka ɲɔgɔn ta ka kɛɲɛ ni SAID suguya ye, nka farigan ka teli ka kɛ a taamasiɲɛ fɔlɔ ye .
• A nafa ka bon ka taa sɔgɔsɔgɔninjɛ furakɛla dɔ fɛ walasa ka bana sɛgɛsɛgɛli ni furakɛli kɛcogo ɲuman sɔrɔ.
• Hali ni furakɛli tɛ se ka bana in furakɛ pewu, a taamasiɲɛw bɛ se ka kunbɛn ka ɲɛ .
• Aw kana aw ɲɛmajɔ a taamasiɲɛw la, barisa u bɛ se ka sugunɛ tiɲɛ ni a ma furakɛ.

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

` SAIDw, sumaya taamasiɲɛw waati ni waati, sumaya segin-segin, farikolojidɛsɛbanaw, sumaya, sumaya denmisɛnw na, jamu banaw