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

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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.