I naute hian sawifiah theih loh khawsik a nei fo em? Hei hi SAIDs (Systemic Autoinflammatory Diseases) vang pawh a ni thei!

I fa te hian khawsik a nei reng em? A chang chuan thla khatah vawi khat emaw vawi hnih emaw khawsik an nei a, doctor-te chuan infection a awm lo, chu chu taksaah virus emaw bacteria emaw a awm lo tih an sawi. Chutiang hunah chuan nu emaw, pa emaw i nih angin hlauhna leh lungkhamna nasa tak i nei a, i rilru a fim hle. "Engvangin nge ka fa chungah hetiang thil hi a lo thlen?" Vawi sangkhat pawh i ngaihtuah tawh ngei ang. Hei hi a chhan chu tun hmaa i la hriat ngai loh thil a ni mai thei. Vawiin hian SAIDs (Systemic Autoinflammatory Diseases) chungchang kan sawi dawn a, hriatthiam harsa leh khawsik a thlen fo thei. Tun hma chuan hengte hi `(Periodic Fever Syndromes)` emaw `(Recurrent Fever Syndromes)` emaw an ti thin.

Heng SAID (Systemic Autoinflammatory Diseases) te hi engte nge ni?

A awlsam zawngin sawi ila, SAIDs hi kan taksa natural immune system a malfunction emaw control problem emaw avanga natna chi khat a ni . Thil thleng chu, pawn lam thil, chu chu virus emaw bacteria emaw awm lovin, taksa hian inflammation a siam mai a ni. Chuvang chuan he khawsik hi a lo thleng fo thin.

Tunah chuan, "Aw, hei hi chutiang Autoimmune Diseases zinga mi a ni em?" Ni lo, heng pahnih hi a danglam deuh. Entirnan, Autoimmune Diseases-ah chuan Rheumatoid Arthritis emaw Lupus ang natnaah chuan kan taksa adaptive immune system hian a cell hrisel tak takte chu a bei sual a ni. Mahse SAID-ah chuan buaina chu innate immune system-ah a awm a ni.

SAID hi Autoimmune Diseases aiin a tlem zawk hle. A tam zawkah chuan hengte hi hereditary , genetic mutation emaw variant emaw avanga lo awm tihna a ni.

Heng SAID te hi a tlangpuiin (mahse a ni lo) i fa chu naute emaw, naupang tê emaw a nih lai aṭangin a intan ṭhin . Naupang hian hun engemaw chen chu natna episode emaw attack emaw a nei ang. Heng episode chhung hian khawsik leh natna dang a lo lang thei a ni. Mahse, heng "attack" inkarah hian naupang chu symptom nei lo a ni thei. SAIDs hi damdawi a awm lo a, mahse a natna lan chhuah dan thunun nan hian enkawlna tha tak tak a awm fo thin.

SAID chi hrang hrangte chu engte nge ni?

Researcher-te chuan SAID chi hrang hrang 60 vel an hmuchhuak tawh a, a aia tam pawh an hmuchhuak mek a ni. A chi hrang hrang hman tlanglawn deuh thenkhat chu hetiang hi a ni:

• Familial Mediterranean fever (FMF): Hei hi genetically determined, recurrent fever syndromes zingah chuan a tam ber a ni. Nausen pum, a rilru leh ruhkawrteah na takin a hring thei a ni.
• Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hei hi naupang tê tê aṭangin a inṭan tlangpui a, a tlangpuiin kum 4 an tlin hmain a ni a, mahse naupang ṭhenkhatah chuan kum 10 an tlin hnuah he natna hi a reh thei a ni.
• Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS): Hei hi naupan lai atanga tleirawl thlengin a awm thei a, a chang chuan puitling thlengin a awm thei bawk.
• Mevalonate kinase deficiency (MKD): Tun hma chuan Hyper-IgD syndrome tia hriat a ni a, hei hi naupang kum khat a tlin hmain a intan tlangpui.
• NLRP3-associated autoinflammatory diseases: Hei hi a hmain Cryopyrin-associated periodic syndromes (CAPS) tia sawi a ni thin. Hemi chhungah hian subtype dang pathum a awm bawk.
• Puitling atanga lo chhuak Still’s disease (AOSD): A hming ang ngeiin he natna hi puitling atanga intan a ni. Systemic juvenile idiopathic arthritis (JIA) tih hming puitling lam chi a ni.
• NOD-2-associated granulomatous disease (Blau syndrome): Hei pawh hi kum 4 an tlin hmain a intan tlangpui a, naupang vun, mit leh ruhkawr a nghawng ber a ni.

SAIDs natna lan chhuah dan chu engte nge ni?

SAIDs natna lan chhuah dan hi naupan lai atanga tan a ni tlangpui. A lan chhuah dan ber leh langsar ber chu periodic emaw episodic fever emaw a ni . A hmaa kan sawi tawh ang khan khawsik awm lohna hun lai hian naupang chu a dam tha thei a ni. Mahse, SAID chi hrang hranga chiang taka lang symptom dang a awm a:

• FMF-ah: Naupang pum, a rilru leh ruhte chu a na hle thei a, a hring thei bawk. A châng chuan, ke hnuai lam emaw, ke ruh emaw-ah vun natna a awm thei bawk.
• PFAPA-ah chuan: Kawng na, hmui ulcer, kawr chhunga lymph node hring. Hengte hi a lan chhuah dan berte an ni.
• TRAPS-ah: Taksa chu a vawt thei a, a khawsik a awm thei bawk. Naupang hian a taksa leh a kut ah taksa ruh natna a nei thei. Rash sen na tak a lo lang thei a, chu chu kut atanga ke thlengin a darh thei a, chumi hnuah taksaah a darh thei bawk.
• MKD-ah: Flu ang chi natna chi hrang hrang i nei thei a, chu chu khawsik, lu na, pum na, ei duh lohna, leh natna dangte a ni.
• NLRP3 natnaah chuan: vun natna, lu na, malaise, ruh natna, leh mit natna (conjunctivitis) te a awm thei.
• AOSD-ah chuan: vun natna, ruh natna, ruh natna. Mi thenkhat chuan ril na te, pum na te, hah te, chak lohna te an nei thei bawk.
• Blau syndrome-ah: Naute hian a kut, ke emaw, a laiah emaw vun natna a nei thei. Tin, ruh na leh mit na an nei thei bawk.

Ngaihtuah teh, Nalini fanu te hian thla engemaw zat kalta atang khan thla tin ni thum atanga ni li chhung chu khawsik sang tak a nei thin. Chumi rual chuan a hmuiah hliam te te a nei bawk a, a kawngah pawh lumps a nei bawk. Doctor hnena a entir chuan PFAPA a ni thei tih a sawi.

SAIDs awm chhante chu engte nge ni?

SAID tam zawk hi chu genetic diseases an ni . Chumi awmzia chu heng syndrome tinte hi kan genes inthlak danglamna avanga lo awm a ni tihna a ni. SAID chi hrang hrang leh a nghawngtu genes te chu hetiang hi a ni:

• FMF: Gene hi `(MEFV)` tih a ni. He gene hian protein `(pyrin)` siam chhuah dan tur a hrilh a ni.
• PFAPA: Hei hi tibuaitu gene dik tak chu hriat chian a la ni lo.
• TRAPS: Gene hi `(TNFRSF1A)` tih a ni. Hetah hian protein `(tumor necrosis factor receptor - TNFR)` siam dan tur thupek chu a lo chhuak a ni.
• MKD: Gene `(MVK)` tih hi a ni. Hei hian `(mevalonic kinase)` tih protein siam turin a hrilh a ni.
• NLRP3 natna: Gene hi `(NLRP3)` tih a ni. Hei hian `(cryopyrin)` tih protein siam dan tur a pe a ni.
• AOSD: Hei hi a chhan dik tak hriat a la ni lo.
• Blau syndrome: Gene `(NOD2)` tih hi a ni. Hei hian protein `(NOD2)` siam chhuah dan tur a hrilh a ni.

Heng genes chungchang chipchiar tak hi i tan chuan a buaithlak deuh mai thei. A awlsam zawngin, heng genes te hian kan taksaa inflammation control tu protein thenkhat siamchhuahna a nghawng a ni. Chuvangin taksa hian chhan awm lovin inflammatory condition a siam thin.

SAIDs hi uluk taka enkawl a nih loh chuan eng nge thleng thei ang?

Heng SAID te hi enkawl dan mumal tak neih a pawimawh hle . A chhan chu taksaa inflammation awm chhunzawm zel chuan `(amyloidosis)` an tih condition a awm thei a ni. Chumi awmzia chu kan kal (kidney)-ah hian protein chi khat a awm tihna a ni a, chu chuan kal (kidney) chu a tichhe vek thei a ni . Chuvangin, symptoms a awm chuan ngaihthah lova damdawi lam thurawn lak a pawimawh hle.

Doctor-te hian engtin nge SAIDs hi an hriat chhuah?

SAIDs hmuhchhuah hi a harsa thei hle a , a chhan chu a natna lan chhuah dan hi natna khirh dang, lupus emaw lymphoma emaw ang chi te nen a inang thei a ni. Chuvangin , i fate dinhmun dik taka hriat leh enkawl turin rheumatologist, doctor, inflammatory diseases lama training bik nei te hmuh a tha ber .

I fa rheumatologist chuan SAIDs hmuhchhuah nan thil engemaw zat a en ang. I fate natna lan chhuah dan leh i chhungte zingah tu emaw chuan khawsik nei fo tawh an awm em tih te a zawt ang che. Doctor chuan he natna hi a ringhlel thei a, chu chu:

• Naupangin khawsik a neih fo chuan .
• Chhungkaw zingah tu emaw chuan khawsik nei fo tawh an nih chuan .
• Naupang chu hetiang khawsik tam zawk nei hnam zinga mi a nih chuan .

Eng test nge hman thin?

Diagnosis finfiah nan i fa rheumatologist chuan test eng emaw zat neih a rawt thei ang. Chung zinga ṭhenkhat chu:

• Lab test: Thisen test `(C-reactive protein - CRP)` leh `(Complete blood count - CBC)` te hian taksaah inflammation a awm leh awm loh enfiah theih a ni. Heng test te hi khawsik i neih chuan `(positive)` a ni a, khawsik a reh chuan a pangngaiah a kir leh thin.
• Urine test: Zun ah hian protein a tam em tih enfiah thin a ni. MKD vei a nih chuan zun-ah hian organic acid ``mevalonic acid'' an tih chu a tam em tih pawh enfiah thin a ni.
• Genetic testing: Genetic testing hian a chunga kan sawi tak genetic variants te hi a enfiah thei a. Mahse, SAID vei naupang thenkhat chuan genetic variant hmuhchhuah hi an nei lo mai thei a, chuvangin test result chu negative a ni thei.

SAIDs enkawlna tur engte nge ni?

SAIDs enkawl dan hi a natna chi leh a nasat danah a innghat a ni . Heng natnate hi damdawi a awm lo va, mahse damdawi hian i fate natna lan chhuah dan chu a thunun thei a ni . Kum khatah i fain attack tlemte chauh a neih chuan a natna lan chhuah dan chu painkiller leh nonsteroidal anti-inflammatory drugs (NSAIDs) hmangin i enkawl thei tlangpui. Mahse, a natna a nasat zawk chuan i doctor chuan enkawlna dang a rawt thei ang:

• FMF: Hei hi damdawi `(Colchicine)` tih hmanga enkawl theih a ni a, inflammation tihziaawm nan. Naupang hnenah `(Colchicine)` pek theih a nih loh chuan `(Canakinumab)` tih damdawi chi khat `(biologic)` i enchhin thei ang.
• PFAPA: Prednisone ang chi steroid hian PFAPA "attack" hun chhung a ti tawi thei a ni. Naupang thenkhat chawm natna damdawi Cimetidine hian a natna lanchhuahna a tiziaawm thei bawk.
• TRAPS: Damdawi `(Canakinumab)` hi TRAPS tan a tha hle. Tin, a natna lan chhuah dan hi doctor-in a pek `(glucocorticoids)` ang chi anti-inflammatory drugs hmanga control theih a ni bawk.
• MKD: `(Canakinumab)` hi MKD enkawlna tha tak a ni bawk. `(NSAIDs)` emaw `(steroids)` emaw hian "attack" laiin a pui thei.
• NLRP3 natna: Immunomodulators Canakinumab, Rilonacept, leh Anakinra te chu NLRP3 nena inzawm natna enkawl nan hlawhtling takin an hmang tawh a.
• AOSD: AOSD enkawlna atan hian anti-inflammatory damdawi chi hrang hrang steroid, disease-modifying anti-rheumatic drugs (DMARDs), leh biologics te hman a ni.
• Blau syndrome: Naupang natna lan chhuah dan azirin immunosuppressants, tumor necrosis factor (TNF) inhibitor, leh/ emaw topical eye medications te hi enchhin theih a ni.

Heng damdawi chungchang chhiar hian i hlauthawng deuh mai thei. Mahse, heng damdawi zawng zawng hi doctor enkawlna nghet tak hnuaia pek vek a ni tih hre reng ang che, naupang tan a tha ber tur angin. Chuvang chuan doctor-in a sawi ang chiaha enkawl dan zawm a pawimawh.

SAIDs status hi a bo dawn em ni?

SAID thenkhat chu dam chhung zawnga awm thin a ni . Mi dangte chu kum engemaw zat an awm thei a , kum upat rualin an bo thei bawk . Thil awm dan thenkhat chu dam chhung zawng a ni a, mahse hun kal zelah chuan attack tamna a tlahniam thei a, symptoms pawh a na lo thei bawk. I doctor chuan i fa dinhmun chiang tak chu a hrilhfiah ang che.

SAID vei naupang enkawl chu thil harsa tak a ni thei. Nangmah ngeiin SAID i neih chuan i thil tawn chu i fa enkawlna kawngah i pui thei ang. I fate chu he dam chhung zawnga dinhmun hi a nunpui theih nan i hriatthiamna pawh a pawimawh hle.

A pawimawh ber chu SAID chungchanga hriatna nei doctor tawnhriat ngah tak takte hnen atanga enkawlna zawn hi a ni. I fate natna lan chhuah dan enkawl kawngah an pui thei a, naupan lai hun tha ber an neih theih nan an pui thei bawk.

Hriat reng tur pawimawh ber berte (Take-Home Message) .

Okay, chuvangin kan sawi tawh atang khan i hriat reng tur pawimawh tak tak thenkhat chu han sawi nawn leh ila:

• I fain khawsik a neih fo chuan, sawifiah theih loh , SAID chhinchhiahna a ni thei.
• SAIDs hi infection vang a ni lo va , taksa hriselna lama harsatna a ni zawk.
• Hengte hi natna tlemte , genetic cause avanga lo awm fo thin an ni .
• SAID chi hrang hrang a zirin symptoms a inang lo thei a, mahse khawsik fo hi a symptom ber a ni .
• Diagnosis leh enkawlna dik tak neih theih nan Rheumatologist pan a pawimawh .
• Enkawlnain natna chu a tidam kim thei lo nain, a lan chhuah dan chu a thunun thei a ni .
• Symptoms te hi ngaihthah suh, enkawl loh chuan kal a tichhia thei a ni .

Hemi chungchanga zawhna dang i neih chuan i chhungkaw doctor emaw, rheumatologist emaw biak hreh suh. Anni chuan an puih belh leh thei ang che.

` SAIDs, periodic fever syndromes, recurrent fever, autoinflammatory diseases, khawsik, naupang khawsik, genetic natna