I fa hian chhan awm lovin khawsik a nei fo em? Heng SAID (Systemic Autoinflammatory Diseases) te hi i zir dawn teh ang!

I fate hian a châng chuan khawsik emaw, natna dang emaw nei lovin khawsik a nei mai mai em? Khawsik chu a lo thleng a, ni engemaw zat hnuah a reh a, ni engemaw zat hnuah a lo lang leh em? Nu leh pa ṭhenkhat chuan chutiang thiltawn chu an hmachhawn a. Chuvangin vawiinah hian hetiang khawpa khawsik chhan awm fo, hmuh harsa tak chungchang kan sawi dawn a ni. Hei hi SAIDs (Systemic Autoinflammatory Diseases) an ti a. Tun hma chuan hei hi `(Periodic Fever Syndromes)` emaw `(Recurrent Fever Syndromes)` emaw an ti thin.

Heng SAID te hi engte nge ni? A awlsam zawngin i hrethiam ang u!

A awlsam zawngin sawi ila, SAIDs hi kan taksa pianpui immune system-a malfunction emaw regulation problem emaw avanga lo awm natna chi khat a ni . Chutiang a nih chuan pawn lam natna (virus emaw bacteria emaw ang chi) awm lovin kan taksa hian inflammation a siam mai a ni. Kan taksa defense system chu overdrive-ah a lut ang mai a ni.

Ngaihtuah teh, kan taksa chhungah sipai rual kan nei (chu chu immune system a ni). An hna chu natna thlen thei hrik an rawn luh hunah do let a ni a, mahse SAID vei taksaah chuan heng sipaite hi a chang chuan an buai mai mai a, taksa chhungah harsatna an siam mai thin.

Tunah chuan hei hi `(autoimmune diseases)` (chu chu autoimmune diseases) ang chi a ni em tih i ngaihtuah mai thei. Entirnan, `(rheumatoid arthritis)` emaw `(lupus)` emaw hi a inang vek. Ni lo, heng pahnih hi a danglam deuh. Autoimmune natnaah chuan thil thleng chu kan taksa acquired emaw 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 natna aiin a tlem zawk hle . A tam zawk chu hereditary , genetic mutation emaw variant emaw avanga lo awm tihna a ni.

SAID hi nausen emaw naupan lai emaw atanga tan a ni tlangpui . Mahse chutiang chu a ni reng reng lo. I fa hian khawsik episode emaw attack emaw a nei thei a, symptom dang a nei thei bawk. I fa chu attack inkar ah symptoms nei lo vek a ni thei. SAID natna hi damdawi a awm lo. Mahse, a tam zawkah chuan natna lan chhuah dan control thei tur enkawlna a awm.

SAID chi hrang hrangte chu engte nge ni?

Tunah hian zirchiangtute chuan SAID chi hrang hrang 60 vel an hmuchhuak tawh a, a aia tam pawh hmuhchhuah mek a ni. A chi hrang hrang hman tlanglawn tak tak thenkhat han thlir ila.

• Familial Mediterranean Fever (FMF): Hei hi genetically inherited, recurrent fever syndromes zingah chuan a tam ber a ni. Naupang pum, a rilru leh a ruhte chu na takin a ti na thei a ni .
• Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hei hi naupang kum 4 an tlin hmain a intan tlangpui a, kum 10 an tlin hnuah pawh he natna hi a takin a reh thei.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Hei hi naupan lai te, tleirawl lai te, a chang chuan puitling lai pawhin a thleng thei.
• Mevalonate kinase deficiency (MKD): Tun hma chuan hyper-IgD syndrome tia sawi thin a ni a, hei hi naute kum 1 hnuai lam atanga tan a ni tlangpui.
• NLRP3-associated autoinflammatory diseases: Hei hi a hmain cryopyrin-associated periodic syndromes (CAPS) an ti thin a. Hemi chhungah hian subtype dang pathum a awm bawk.
• Puitling atanga lo chhuak Still’s disease (AOSD): Hei hi puitling atanga lo chhuak systemic juvenile idiopathic arthritis (JIA) a ni.
• NOD-2-associated granulomatous disease (Blau syndrome): Hei 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? Engtin nge kan hriat chian?

SAIDs symptoms hi naupan lai atanga tan a ni tlangpui. A lan chhuah dan tlangpui leh langsar ber chu periodic emaw episodic fever emaw a ni . Mite chu attack inkarah symptom nei lovin an awm tlangpui. Mahse, SAID chi hrang hrangah hian symptom dang bik a awm thei.

A chunga kan sawi tak SAID chi hrang hrang chhinchhiahna chiang tak takte hi han en ila:

• FMF: Hei hian naupang pum, a rilru leh ruhteah natna nasa tak a thlen thei a, chubakah inflammation a thlen thei bawk . A châng chuan, ke hnuai lam emaw, ke ruh emaw-ah hian vun a lo awm thei bawk. Ngaihtuah teh, naupang pakhat chu a pum na leh khawsik a nei nghal a. Nu leh pate an lungkham a, damdawi ina an hruai chuan appendicitis niin an ngai mai thei. Mahse, chutah chuan a reh a, ni engemaw zat hnuah a rawn let leh ta a ni.
• PFAPA: Hei hian natna lan chhuahna a thlen thei a, chu chu ril na, hmui na, leh kawr chhunga lymph node hring te a ni .
• TRAPS: Hei hian khawsik a thlen thei a, rilru leh kut ruh na , leh kut leh ke atanga intan leh taksa pum huapa darh chhuak, vun sen na tak a thlen thei bawk.
• MKD: Hemi kawngah pawh hianI rilru a hahdam lo thei a, khawsik, lu na, pum na, ei duh lohna, leh flu ang chi natna te a awm thei.
• NLRP3 natna: Hengte hian vun natna, lu na, malaise, ruh natna, leh mit sen (conjunctivitis) te a thlen thei a ni.
• AOSD: Hei hi vun natna, ruh natna, leh ruh natna thlentu tlangpui a ni . Mi thenkhat chuan ril na te, pum na te, hah lutuk te, taksa na te an nei thei bawk.
• Blau syndrome: Hei hian i naute kut, ke, emaw taksaah vun natna a thlen thei . Tin , ruh natna leh mit natna a thlen thei bawk .

Engvangin nge heng SAID te hi a lo awm? A chhante chu engte nge ni?

SAID tam zawk hi chu genetic diseases an ni . Chu chu, syndrome tin hi gene bik pakhata danglamna (variant) avanga lo awm a ni tihna a ni.

SAID chi hrang hrang lian tak tak nghawng thei genes te hi han en ila:

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

Heng SAID te hian harsatna dang a thlen thei em?

Ni e, hei hian enkawl ṭhat loh chuan harsatna a thlen thei . Taksaah inflammation a awm chhunzawm zel chuan ``amyloidosis`` tih natna a thlen thei a ni.`` Chu chu kan kal (kidney)-ah protein chi khat a awm tihna a ni. Hei hian kal (kidney) a tichhe vek thei a ni . Chuvang chuan natna hi hriat hmasak a, enkawlna lak a pawimawh hle.

Engtin nge doctor hian he SAIDs condition hi a hriat theih ang?

In fact, diagnosing these autoinflammatory diseases can be a bit challenging , an symptoms hian lupus ang chi natna khirh tak dang emaw, lymphoma ang chi cancer emaw a entawn thei a ni. Chuvangin, i fate dinhmun dik tak hriatchhuah leh enkawl turin doctor, inflammatory diseases lama training bik nei, rheumatologist pan a pawimawh hle .

I fate rheumatologist chuan SAIDs a hriat theih nan thil engemaw zat a hmang ang. I fa natna lan chhuah dan leh i chhungkuaa tu pawhin heng natna hi an nei em tih te a zawt ang che (biological family history). Doctor chuan i fa hian periodic fever syndrome a nei thei tih a ringhlel thei a, chu chu:

• Naupangin khawsik a neih fo chuan .
• Chhungkaw zingah hetiang hian khawsik nei fo tawh an awm a nih chuan .
• Naupang chu hetiang ang periodic fever vei duh pawl zinga mi a nih chuan .

Hemi atan hian eng test nge tih a nih?

Diagnosis finfiah nan i fa rheumatologist chuan test eng emaw zat neih a rawt thei ang. Chûng zîngah chuan:

• Lab test: ``C-reactive protein (CRP)`` leh ``complete blood count (CBC)`` tih ang chi test hmangin taksaa inflammation awm leh awm loh enfiah theih a ni. Heng test te hi fever "attack" laiin positive an ni a, "attack" a zawh chuan normal ah an kir leh thin.
• Urine test: Urine protein test hmangin zun chhunga protein tam lutuk a awm leh awm loh enfiah theih a ni. MKD-ah chuan zun test-a organic acids (carbon atom pakhat awmna acid) en chuan mevalonic acid level sang tak a awm thei a ni.
• Genetic testing: Hei hian genetic variant a awm leh awm loh a enfiah thei. Mahse, SAID vei naupang thenkhat chuan genetic variant hmuhchhuah hi an nei lo mai thei a, hei hian test result negative a thlen thei a ni.

Heng SAIDs te hi eng enkawl dan nge ni?

SAIDs enkawl dan hi a natna chi leh a nasat dan azirin a inang lo . Heng natnate hi damdawi a awm lo. Mahse , i fate symptoms chu damdawi hmangin control theih a ni tlangpui . Kum khatah i fain attack tlemte chauh a neih chuan an natna lan chhuah dan tihtlem nan nonsteroidal anti-inflammatory drugs (NSAIDs) ang chi natna tidamtu i pe thei mai thei. Mahse, a natna a nasat zawk chuan i doctor chuan enkawlna dang a rawn rawt thei a ni.

Chung zinga ṭhenkhat chu hetiang hi a ni:

• FMF: Hei hi control nan hian damdawi `(colchicine)` tih hi hman theih a ni. Hei hian inflammation a ti tlem a ni. Naupangin colchicine a ei theih loh chuan `(biologic)` chi damdawi `(canakinumab)` an tih chu enchhin theih a ni.
• PFAPA: PFAPA "attack" hun chhung tih tawi nan steroid (a tlangpuiin prednisone) pek theih a ni. Naupang thenkhatah chuan chaw kawng ulcer enkawlna atana hman thin cimetidine hian natna lanchhuahna control nan a pui thei bawk.
• TRAPS: Hei atan hian damdawi ``canakinumab'' an tih hi a tangkai fo thin. Tin, doctor-in a pek anti-inflammatory damdawi, ``glucocorticoids'' te hmangin natna lanchhuahna chu a reh thei bawk.
• MKD: 1.1.Canakinumab hi MKD damdawi tha tak a ni bawk. "Attack" laiin NSAID emaw steroid emaw pek pawh a \angkai thei hle.
• NLRP3 natna: Hemi atan hian immunomodulators canakinumab, rilonacept, leh anakinra te chu hlawhtling takin hman a ni.
• AOSD: AOSD tan hian anti-inflammatory damdawi chi hrang hrang hman a ni. Entirnan, steroid, natna tidanglam thei anti-rheumatic drugs (DMARDs), leh biologics te hi a ni.
• Blau syndrome: I fate natna lan chhuah dan azirin immunosuppressants, tumor necrosis factor (TNF) inhibitor, leh/ emaw topical eye medications te an hmang thei ang.

Heng SAIDs condition te hi a awm reng em? Nge an kal bo dawn em ni?

Autoinflammatory condition thenkhat chu dam chhung zawng a ni . Mi dangte chu kum engemaw zat an awm thei a, kum upat deuh deuhin an bo thei bawk. Thil awm dan thenkhat chu dam chhung zawng a ni a, mahse hun kal zelah chuan attack tamna leh nasat dan a tlahniam thei. I fa doctor chuan i fa dinhmun chungchang chiang takin a pe thei che a ni.

Naupang autoinflammatory disease nei enkawl chu thil harsa tak a ni thei. SAID i neih chuan i mimal thil tawn te hmangin i fa enkawlna chu i thlawp tha zawk thei ang. I thil tawnte chu i fa chu he damchhung dinhmuna a insiamrem theih nân i hmang thei bawk.

A pawimawh ber chu SAID chungchanga hriatna nei specialist tawnhriat ngah tak takte hnen atanga enkawlna lak a ni. Chutiang doctor-te chuan i fate natna lan chhuah dan enkawl kawngah an pui thei a, naupan lai hun ṭha ber an neih theih nan an pui thei bawk.

Take-Home Thuchah hnuhnung ber

Chuvangin, vawiin a kan sawi SAID chungchangah hian ngaihdan i neih ka beisei. I fain a khawsik fo, sawifiah theih loh a neih chuan, chu mi chungchâng chu doctor hnênah sawipui ngei ang che , tih hre reng ang che . Thil pangngai anga ngaiin ngaihthah mai mai suh.

• SAIDs hi natna chi khat, khawsik thlentu tam tak, taksa hriselna lama harsatna avanga lo awm thin a ni.
• Chungte chu genetic a ni fo a, kum naupan lai atanga tan a ni.
• Hei hi damdawi nghet awm lo mah se, a natna lan chhuah dan thunun thei damdawi a awm.
• Diagnosis dik tak leh specialist doctor (Rheumatologist) hnen atanga enkawl a pawimawh hle.
• Enkawlna hi tan hmaa tan a nih chuan amyloidosis ang chi complication te chu ven theih a ni a, naupang chu nun pangngaiin a nung thei bawk.

Hemi chungchanga zawhna dang i neih chuan i chhungkaw doctor emaw naupang enkawltu emaw zawt hreh suh.

` SAIDs, Systemic Autoinflammatory Diseases, Khawsik tam tak, Genetic Diseases, Naupang khawsik, Immune System, Rheumatologist