I fa hian khawsik a nei fo em? SAIDs (Systemic Autoinflammatory Diseases) vang a ni thei ang em?

Aw, tunlai hian i naupang te hian khawsik a nei fo em? I dam leh tawh nia i ngaih lai tak chuan a rawn lang leh ta em ni? A chang chuan hei hi a chhan chu virus emaw bacteria tlanglawn tak a ni lo thei bawk. Vawiin hian condition pakhat kan sawi dawn a, a buaithlak deuh a, mahse hriat a pawimawh hle. Hei hi SAIDs an ti a, chu chu Systemic Autoinflammatory Diseases tihna a ni. Tun hma chuan hei hi `(Periodic Fever Syndromes)` emaw `(Recurrent Fever Syndromes)` emaw an ti thin.

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

A awlsam zawngin, SAIDs hi kan taksa pianpui immune system-a malfunction emaw regulatory problem emaw avanga lo awm natna chi khat a ni . Hei vang hian naupang chu natna hrik (virus emaw bacteria emaw ang chi) nei lo pawhin khawsik a nei fo thei a ni.

A pawimawh ber chu heng SAIDs an tih te hi `(Autoimmune Diseases)` (entir nan `(Rheumatoid Arthritis)` emaw `(Lupus)` emaw i hriat tawh mai thei. `(Autoimmune)` natnaah chuan kan taksa `(adaptive immune system)`-a tihsual engemaw zat a awm avangin, chu system chuan a cell hrisel tak takte chu a bei thin. Mahse SAID-ah chuan thil danglam tak a thleng thin.

SAID hi autoimmune natna aiin a tlem zawk hle. Anni hi rochun atanga lo piang an ni fo a , genetic mutation avanga lo awm an ni .

A tlangpuiin, mahse a ni lo, SAID symptoms hi naupang a naupan lai atanga intan a ni a, naute emaw, naupang tê emaw a nih lai pawhin . I fa hian he natna nen hian "periods" a nei thei a, khawsik leh symptom dang a nei thei bawk. Heng hun inkar chhung hian i fa hian symptom a nei lo thei. SAIDs hi damdawi awm lo mah se , i fate natna lan chhuah dan thunun thei tur enkawlna 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 thar pawh hmuhchhuah a la ni reng a ni. SAID chi hrang hrang hman tlanglawn tak tak thenkhat chu hetiang hi a ni:

• Familial Mediterranean fever (FMF): Hei hi genetically inherited, recurrent fever syndromes zingah chuan a tam ber a ni. Naupangte pum, rilru leh ruh na tak tak a thlen thei a ni.
• Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Hei hi naupang kum 4 an tlin hmain a intan tlangpui a, a chang chuan kum 10 an tlin hnuah pawh a takin a dam thei a ni.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Hei hi naupan lai te, tleirawl lai te, puitling lai pawhin a thleng thei.
• Mevalonate kinase deficiency (MKD): Tun hma chuan ``Hyper-IgD syndrome'' tia sawi thin a ni a, hei hi naupang kum khat a tlin hmain a intan tlangpui.
• NLRP3 nena inzawm autoinflammatory natna: 1.1.Hei hi a hmain `(Cryopyrin-associated periodic syndromes - CAPS)` an ti thin. Hemi chhungah hian subtype dang pathum a awm bawk.
• Puitling atanga lo chhuak Still’s disease (AOSD): A hming ang bawkin hei hi puitling atanga lo chhuak naupangte natna ``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?

SAIDs symptoms hi naupan lai atanga tan a ni tlangpui. A lan chhuah dan ber leh langsar ber chu periodic fever a ni . Khawsik lai hian mi an hrisel thei tlangpui a, natna lanchhuahna an nei lo. Mahse, SAID chi hrang hranga chiang taka awm symptom dang a awm thei bawk. Eng nge an nih i en ang u:

• FMF: Hei hian naupang pum, a rilru leh ruhte a ti na thei a, a na hle bawk . Ke hnuai lam emaw, ke ruh emaw-ah pawh vun a awm thei bawk.
• PFAPA: Hei hian rilru na, hmui na, leh kawr chhunga lymph node hring te a thlen thei . Entirnan, i fain tonsillitis leh hmui ulcer a neih fo ang chi symptoms a neih chuan PFAPA a ni thei.
• TRAPS: Hei hian trunk/torso leh arms ah chills leh muscle na a thlen thei . Tin, kut leh ke atanga intan, taksa chhunga darh chhuak, vun sen na tak a siam thei bawk.
• MKD: Hei hian natna lan chhuahna a thlen thei a, chu chu khawsik, lu na, pum na, ei duh lohna, leh flu ang chi natna a thlen thei a ni .
• NLRP3 natna: He natna hian vun natna, lu na, malaise, ruh natna, leh mit/conjunctivitis pink a thlen thei a ni.
• AOSD: Hei hi vun natna, ruh natna, leh ruh natna te a ni . Mi thenkhat chuan rilru na, pum na, hahna, leh malaise te pawh an nei thei bawk.
• Blau syndrome: Hei hian i naute kut, ke, emaw, midsection emaw-ah vun natna a thlen thei . Tin , ruh natna leh mit natna a thlen thei bawk .

SAIDs awm chhante chu engte nge ni?

SAID tam tak hi genetic diseases an ni . Chu chu, gene bik pakhata mutation (variant) bik chuan syndrome tin a thlen tihna a ni. Kan taksa hian hna tin tih dan tur min hrilhtu instruction (gene) set khat a nei tih han ngaihtuah teh, chu lehkhabuah chuan letter pakhat a dik lo a nih chuan hna dang zawng zawng chu a kal sual thei a ni.

SAID chi hrang hrang leh a kaihhnawih genes te chu hetiang hi a ni:

• FMF: 1.1.Gene `MEFV` hi protein `(pyrin)` siam dan tur hrilhfiahna petu a ni.
• PFAPA: Hei hi a chhan dik tak hi hriat chian a la ni lo .
• TRAPS: Gene `TNFRSF1A` tih hi a ni. Hei hian `(tumor necrosis factor receptor - TNFR)` tih protein siam turin a hriattir a ni.
• MDK: Gene chu `MVK` tih a ni. Hei hian `(mevalonic kinase)` tih protein siam turin a hrilh a ni.
• NLRP3 natna: Gene `NLRP3`. Hei hian `(cryopyrin)` tih protein siam turin a hrilh a ni.
• AOSD: Hei hi a chhan dik tak hi hmuhchhuah a la ni lo .
• Blau syndrome: Gene `NOD2` tih hi a ni. Hei hian `(NOD2)` tih protein siam chhuah dan tur a hrilh a ni.

Hengte hi genetic factors avanga lo awm a nih chuan nu leh pa sualna a ni lo tih hriat a pawimawh. Hengte hi biological process complex tak tak an ni.

SAIDs avanga harsatna awm thei

Heng natnate hi enkawl dan dik tak neih a pawimawh hle . A chhan chu, taksaah inflammation a awm chhunzawm zel chuan `(Amyloidosis) tih natna a thlen thei a ni. `(Amyloidosis)` hi kal chhunga protein awm khawm a ni . Hei hian kal (kidney) a tichhe vek thei a ni . Chuvangin natna lanchhuahna a awm chuan damdawi lam pan vat a pawimawh.

SAID te hi engtin nge an hriat chhuah?

Dik tak chuan SAIDs hmuhchhuah hi a harsa deuh thei 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 tak hriatchhuah leh enkawl dan tur , inflammatory diseases chungchanga special training nei doctor (Rheumatologist) pan a pawimawh hle .

I fate rheumatologist chuan SAIDs hi a hriat theih nan thil engemaw zat a hmang ang. I fate natna lan chhuah dan leh i chhungkuaa tu emaw chuan heng natna hi an nei tawh em tih a zawt ang (biological family history). Doctor chuan i fa hian Periodic Fever Syndrome a nei tih a ringhlel thei a, chu chu:

• Khawsik i neih fo chuan
• Chhungkaw zingah hetiang hian periodic fevers nei tawh an awm a nih chuan
• Chutiang khawsik vei duh pawl zinga mi i nih chuan

Eng test nge hman thin?

Doctor chuan a natna hriat chian nan test eng emaw zat neih a rawt thei a ni. Chûng zîngah chuan:

• Lab test: `(C-reactive protein - CRP)` leh `(Complete Blood Count - CBC)` ang chi test te hian taksaah inflammation a awm leh awm loh enfiah theih a ni. Heng test te hi khawsik a awm chuan `(positive)` an ni a, khawsik a reh chuan a normal value-ah an kir leh thin.
• Zun test: 1.1.Urine protein test hmangin zun chhungah protein a tam em tih enfiah theih a ni. MKD-ah chuan zun test-naah organic acids a awm leh awm loh enfiah chuan mevalonic acid level a sang hle ang.
• 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. Chuvangin test result chu ``negative`` ni mahse SAID a awm theihna tur chu a awm thei lo.

SAIDs hi engtin nge enkawl an nih?

SAIDs enkawl dan hi natna chi leh nasat dan azirin a inang lo . Heng natnate hi tihdam kim theih ni lo mah se, naupang natna lan chhuah dan chu damdawi hman hmangin a thunun tha thei a ni . Entirnan, i fain kum khatah vawi engemaw zat chauh heng khawsik hi a vei a nih chuan, natna tidamtu leh nonsteroidal anti-inflammatory drugs (NSAIDs) te hian a natna lan chhuahna chu a thunun thei tlangpui. Amaherawhchu, natna hi a na zawk a nih chuan doctor chuan enkawl dan dang a rawn rawt thei a ni.

Chutiang enkawlna thenkhat chu hetiang hi a ni:

• FMF: Hei hi damdawi `(Colchicine)` tih hmanga enkawl theih a ni a, chu chuan inflammation a tiziaawm thei a ni. `(Colchicine)` i ei thei lo a nih chuan `(Biologic)` chi khat `(Canakinumab)` tih hi i enchhin thei ang.
• PFAPA: PFAPA flare-ups hi steroid (a tam zawkah chuan prednisone) pek hian a ti tawi thei a ni. Naupang thenkhat chuan an natna lan chhuah dan chu an thunun thei a, chu chu chawhmeh natna enkawlna atana hman thin Cimetidine an hmang a ni.
• TRAPS: Damdawi `(Canakinumab)` hi TRAPS tan a tha hle. Tin, prescription anti-inflammatory medications `(Glucocorticoids)` te pawh hian symptoms a tiziaawm thei bawk.
• MKD: `(Canakinumab)` hi MKD enkawlna atan duhthlan tur tha tak a ni. Khawsik lai hian `(NSAIDs)` emaw `(Steroids)` emaw hman a \angkai thei bawk.
• NLRP3 natna: Immunomodulators dang Canakinumab leh Rilonacept, Anakinra te hi NLRP3 natna enkawl nan a tha hle.
• AOSD: AOSD tan hian anti-inflammatory damdawi chi hrang hrang hman a ni. Chung zingah chuan `(Steroids)`, `(Natna tidanglam thei Anti-Rheumatic Drugs - DMARDs)`, leh `(Biologics)` te an tel.
• 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.

SAIDs condition hi a mah chauhin a tha chho em?

SAID thenkhat chu dam chhung zawng a daih thei . Mi dangte chu kum engemaw zat an awm thei a, kum upat deuh deuhin an ṭha chho thei bawk. Thil awm dân ṭhenkhat chu dam chhûnga awm a nih laiin, hun kal zêlah khawsik tamna chu a tlahniam thei a, a lan chhuah dân pawh a na lo thei bawk . I doctor chuan i fa dinhmun bik chungchangah chipchiar takin a pe thei che a ni.

SAID vei naupang enkawl chu thil harsa tak a ni thei. Nangmah ngei pawhin SAID i nei tawh mai thei, mahse i mimal thil tawnte hmangin i fa enkawl dan tur leh he dam chhung zawnga an dinhmun hi an hmachhawn theih nan i pui thei ang.

A pawimawh ber chu SAIDs intricacies hre chiang tak specialist te hnen atanga enkawlna lak 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 hian i hriat reng tur thenkhat chu khaikhawm ila.

• I fain a khawsik fo, sawifiah theih loh a neih chuan , chu chu doctor nen sawiho rawh. SAID chhinchhiahna a ni thei.
• SAIDs hi innate immune system -a harsatna a ni a, natna hrik kai tlangpui a ni lo.
• Hengte hi ``Autoimmune'' natna nen a danglam a ni .
• A tam zawkah chuan hengte hi genetic factors avanga lo awm a ni .
• Diagnosis dik tak neih theih nan Rheumatologist pan a pawimawh .
• Enkawlna hian symptoms a control tha hle .
• Kal a chhiat loh nan enkawl hmasak a pawimawh .

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

` SAIDs, hereditary fever, khawsik fo, naupang khawsik, immune system, genetic diseases, rheumatologist