I naute hian chhan awm lovin khawsik a nei fo em? Heng SAID (Systemic Autoinflammatory Diseases) te hi han sawi ila

I fa te hian khawsik a nei reng mai em? Ni engemaw zat a khawsik hnuah a dam leh a, ni engemaw zat hnuah a khawsik leh chauh a ni em? Virus emaw bacteria emaw infection tel lovin engvangin nge hetiang hi a awm tih i ngaihtuah mai thei. Hei hi SAIDs (Systemic Autoinflammatory Diseases) , mi tam takin an hriat ngai loh natna hmuh tur awm lo vang a ni thei. Ngaihtuah buai suh, thu awlsam takin sawi ila.

SAID tih hi eng nge ni? A awlsam zawngin i hrethiam ang u.

A awlsam zawngin sawi ila, SAIDs hi natna chi khat, natna hrik (virus emaw bacteria emaw ang chi) nei lo, khawsik lo awm fo, lo lang leh thin a ni. Hei hi a chhan chu kan fa pianpui immune system a thawk tha lo vang a ni . Hei hi a hmain ‘Periodic Fever Syndromes’ an ti thin.

Tunah chuan i ngaihtuah mai thei, hei hi ‘autoimmune’ natna a ni em? Autoimmune natna Rheumatoid Arthritis emaw Lupus emaw ang chi kan hre fo thin. Ni lo, hei hi a danglam a ni.

Kan taksa chhungah hian defence army kan nei a, kan immune system hi han ngaihtuah teh. He sipai hian hmunpui pahnih a nei a:

1. Innate Immune System: Hei hi kan pianpui sipai a ni a, eng hrik pawh bei hmasa bertu a ni.

2. Adaptive Immune System: Hei hi kan lo puitlin ruala hrik chi hrang hrang do tura a bik taka training nei sipai a ni.

SAID-ah chuan buaina chu innate immune system -ah a ni . Chu immune system chu a buai a, taksa chhungah hmelma awm lovin insual a tan ta a ni. He inbeihna rah chhuah chu taksaah inflammation leh khawsik a ni.

Mahse kan sawi tawh autoimmune diseases-ah chuan buaina chu a hnua training thin adaptive army-ah a ni. Chu sipai chuan a taksa chhunga cell tha awmte chu a hre thei lo va, a bei tan ta a ni.

SAID hi autoimmune natna aiin a tlem zawk hle. Genetic cause avanga lo awm an ni fo a, chu chu rochun an ni tihna a ni. Heng symptoms te hi naupang a naupan lai atanga tan a ni tlangpui a, nausen lai emaw, naupan lai emaw a ni. Naupang chuan natna episode emaw attack emaw a nei nghal a, khawsik leh natna dang a nei a, chu chu ni engemaw zat chhungin a reh tlangpui. Naupang chu a damloh hunah a hrisel famkim thei a ni. Damdawi awm lo mah se, a natna lan chhuah dan thunun thei tur enkawlna tha tak tak a awm.

SAID chi hrang hrangte chu engte nge ni?

Tun thlengin zirchiangtute chuan SAID chi hrang hrang 60 vel an hmuchhuak tawh a, chi thar hmuhchhuah a ni reng bawk. Heng chi hrang hrang zingah hian a tlangpuiin kan rawn tarlang e.

Heng natna lan chhuah dan tlangpui chu engte nge ni?

SAIDs natna lan chhuah dan ber leh langsar ber chu recurrent fever a ni . Mahse, khawsik bakah hian natna chi hrang hrang a zirin symptom dang pawh a awm thei bawk.

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

Kan sawi tawh ang khan SAID tam tak hi genetic diseases an ni . Chumi awmzia chu heng natnate hi gene pakhata mutation (variant) avanga lo awm a ni tihna a ni.

Enkawlna mumal tak an dawn loh chuan eng nge thleng thei ang?

Hei hi thil pawimawh tak a ni. Hetiang dinhmun hi enkawl dan dik tak hmanga control a pawimawh hle. A chhan chu, hetiang hian taksaah hian inflammation a awm chhunzawm zel a nih chuan Amyloidosis tih natna a thlen thei a ni. A awlsam zawngin kan kal (kidney)-ah hian protein chi khat a awm thei a, chu chuan kal (kidney) chu a tichhe vek thei a ni. Chuvang chuan natna hriat chian a, enkawl vat a pawimawh hle.

He natna hi engtin nge doctor hian a hriat theih ang?

Dik tak chuan SAIDs hmuhchhuah hi a harsa deuh thei a, a chhan chu natna khirh tak dang, lupus ang chi natna lan chhuah dan nen a inang thei a ni.

Chuvang chuan, hetiang natna chi hrang hranga specialist doctor pan hi a pawimawh ber a ni. Doctor chu ruh leh taksa hriselna nena inzawm natna hrang hranga tui tak chu Rheumatologist an ti a .

I doctor chuan diagnosis siam nan thil engemaw zat a ngaihtuah ang. I fa natna lan chhuah dan leh chhungkaw history-a he natna hi a awm leh awm loh a zawt mai thei. I doctor chuan SAIDs a ringhlel thei a, a bik takin:

• Naupangin khawsik a neih fo chuan.
• Chhungkaw zingah hian hetiang flu ang chi natna lo awm leh tawh an awm chuan.
• Hêng natnate hi hnam ṭhenkhat zîngah a hluar avângin chu pawh chu ngaihtuah a ni.

Eng test nge tih thin?

Doctor chuan natna hriat chian nan test eng emaw zat neih a rawt mai thei.

• Thisen test (Lab tests): C-reactive protein (CRP) leh CBC (Full Blood Count) ang chi test te hian taksaah inflammation a awm leh awm loh enfiah theih a ni. Heng level te hi damloh laiin a pung a, taksa a dam chuan a normal level ah a kir leh thin.
• Urine test: Zun chhunga protein level sang leh sang a awm leh awm loh enfiah thin. Natna thenkhat, MKD ang chiah hian zun test hian acid bik a hmuchhuak thei a.
• Genetic testing: He test hian genetic mutations a hmuchhuak thei a ni. Mahse, a chang chuan naupangin SAID a nei thei a, mahse genetic test-ah erawh chuan a hmuchhuak lo mai thei. Chuvangin, test result negative pawh nise, 100% in natna a awm lo tih hi sawi theih a ni lo.

Hei hi eng enkawlna nge ni?

SAIDs enkawl dan chu natna chi leh nasat danah a innghat a ni. Heng natnate hi tihdam kim theih ni lo mah se, damdawi hian nasa takin a natna lan chhuah dan a thunun thei a ni.

• FMF: Hemi atan hian damdawi Colchicine an tih hi hman a ni. Taksa a inflammation a tihziaawm. Chu damdawi chuan naupang tan a thawk tha lo a nih chuan Canakinumab ang damdawi lam an hawi thei a ni.
• PFAPA: Prednisone ang chi steroid hi hun rei lote chhung pek theih a ni a, chu chuan natna rei zawng a tihtlem thuai a ni.
• TRAPS: Canakinumab tih hi a niDamdawi hi a hlawk hle. Tin, anti-inflammatory drugs glucocorticoids ang chi te pawh hman a ni bawk.
• MKD: Hemi atan hian damdawi Canakinumab pawh a tangkai hle. Natna lai hian NSAID (painkillers) emaw steroid emaw hian a pui thei a ni.
• NLRP3 Natna: Hemi atan hian Immune-modifying drugs Canakinumab , Rilonacept , leh Anakinra te chu hlawhtling takin hman a ni.
• Blau Syndrome: A natna lan chhuah dan azirin immunosuppressant, TNF inhibitor, leh mit damdawi te hman theih a ni.

He natna hi a dam vek dawn em ni?

SAID thenkhat chu dam chhung atan a ni. Mahse, ṭhenkhat chu PFAPA ang bawkin naupang a lo puitlin chuan anmahni chauhin an bo mai thei. Nun chhung zawnga awm thei ni mahse, hun kal zelah natna vei tam dan leh a lan chhuah dan nasat dan chu a tlahniam thei a ni. I doctor chuan i fa dinhmun chungchang chipchiar takin a pe ang che.

Hetiang natna nei naupang enkawl chu thil harsa tak a ni thei. Mahse specialist dik takte puihnain i fate natna lan chhuah dan chu i enkawl thei a, naupan lai hun tha ber i pe thei bawk.