
dermatomyocytis

dermatomyocytis (dii. emm.) haah eka dirghakalin dahak vikar aahe joou tvacha aanee snayunvar parinam karato. samanyatah tvachevar pural padane aanee kalantarane snayunmadhye kamjori vadhane hii tyachi lakshane aahet. heey achanak houu shakatat kinwa kahee mahinyant vikasit houu shakatat. itar lakshananmadhye vajan qamee hone, taap, fuffusanchi jaljal kinwa prakash sanvedansheelata yancha samavesh asoo shakato. guntaguntinmadhye snayu kinwa tvachemadhye calcium sathavane samavisht asoo shakate.
kaaran adnyat aahe. siddhantanmadhye asey samavisht aahe qee haah eka svayampratikar rog aahe kinwa vishanujanya sansargacha parinam aahe. dermatomyocytis haah anek prakarchya durbhavnashi sambandhit parenoplastic syndrome mhanoon vikasit houu shakato. haah eka prakarcha dahak myopathy aahe. nidan heey samanyatah kahee lakshane, rakt chachanya, vidyut-parikshan aanee snayunchya biopsychya sanyogavar aadharit asate.
yaa sthitivar konatahi upchar maheet nasla taree, upcharanmule samanyatah lakshane sudharatat. upcharanmadhye aushadhe, sharirik upchar, vyayam, ushma upchar, orthotics aanee sahayyak upkarane aanee vishranti yancha samavesh asoo shakato. jarr steroids changle kaam karat nastil tarr corticosteroids kutumbatil aushadhe samanyatah methotrexet kinwa ezathioprene sarkhya itar ghatkansah vaparali jaatat. antahshira immunoglobulin dekhil parinam sudharu shakate. bahutek lock upcharane sudharatat aanee kahee lokanmadhye hii sthitee purnapane burry hote.
darvarshi darr 100,000 lokanpaiki eka jann navyane prabhavit hoto. hii sthitee samanyatah 40 aanee 50 chya dashkatil lokanmadhye udbhavate, jyat purushanpeksha mahilanna jaast vela parinam hoto. tathapi, konatyahi vayogatatil lock prabhavit houu shakatat. yaa sthitiche varnan pahilyanda 1800 chya dashakat karnyat aale hote.
chinhe aanee lakshane.
mukhya lakshananmadhye donhi varchya hatanmadhye kinwa mandimadhye snayu kamkuvat honyasah tvachevar pural padanyache anek prakar samavisht aahet.
tvacha.
puralanchya eka prakarala 'heliotrope' (jambhala rung) kinwa lealack mhanatat, parantu too laal dekhil asoo shakato. heey soojasah dolhyanchya bhovati houu shakate, parantu chhatichya varchya bhagavar kinwa pathivar dekhil houu shakate jyala stananchya varr 'shaal' (manebhovati) kinwa 'vhee-sine' mhanatat aanee chehara, varche haat, mandee kinwa hatanvar dekhil houu shakate. puralanchya aanakhi eka prakarala 'gotronche chinh' mhanatat, jeey laal kinwa jambhale asatat, kahivela koradya, kinchit unchavalelya popules asatat, jyaa botanchya konatyahi sandhyanvar (metacarphophalengeal sandhe kinwa interfalengeal sandhe) futatat. gotronche guthalya kopar, gudghe kinwa payansah itar hadanchya pramukh bhaganvar dekhil aadhalu shakatat. heey serve pural suryaprakashachya samparkat aalyane adhik kharab hotat aanee anekada khoop khaaj, vednadayak asatat aanee raktastrav houu shakato.
jarr ekhadya vyaktila kamkuvatpana kinwa asamanya snayu enzymeshivay keval dii. emm. chey vaishishtyapurn tvacheche nishkarsh disun aale, tarr tyala kinwa teela amyopathic dermatomyocytis (aii. dii. emm.) anubhav yet asel, jyala purvi "dermatomyocytis sine myocytis" mhanoon olakhale jaat asey.
snayu.
dii. emm. asalelya lokanna nikatvarti snayunmadhil (udaharanarth, khande aanee mandee) snayuncha kamkuvatpana haloohaloo bighadat jaato. yaa snayuncha vapar karnari kameh basane, uchalane aanee payya chadhane, dermatomyocytis asalelya lokansathi adhikadhik katheen houu shakate.
itar.
sumare 30 takke lokanna soojalele, vednadayak sandhe aahet, parantu heey samanyatah saumya asate.
kahee lokanmadhye, yaa sthiticha fuffusanvar parinam hoto aanee tyanna khokala kinwa shwas ghenyas tras houu shakato. jarr rog hrudayavar parinam karat asel tarr erythmia houu shakato. jarr teey potatil kinwa aatadyanmadhil raktavahinyanvar parinam karat asel, jeey kishoravayin dii. emm. madhye adhik samanya aahe, tarr lokanna raktachya ulatya houu shakatat, kalya tarry aantranchya halchali houu shakatat kinwa tyanchya jee. aay. margat kuthetari chhidra houu shakate.
dermatomycytismule aanakhi guntagunt honyachi shakyata aahe. yaa guntaguntinmadhye annanaliketil snayunvar parinam jhalyamule gilanyas adchan yene samavisht aahe jyamule kuposhan houu shakate aanee ann kinwa dravpadarth fuffusanmadhye shwas ghenyas karanibhoot tharoo shakatat.
karane.
kaaran adnyat aahe, parantu heey prarambhik vishanujanya sansarg kinwa karkarogamule houu shakate, jyapaiki eka svayampratikar pratisad vadhavu shakate.
dermatomyocytischi 7 teey 30 takke prakarane karkarogamule udbhavtat, bahudha svayampratikar pratisad mhanoon. andashayacha karkarog, stanacha karkarog aanee fuffusacha karkarog heey sarvat samanyapane sambandhit karkarog aahet. amyopathic dii. emm. asalelya 18 teey 25 takke lokanna dekhil karkarog aahe. dermatomyocytischya sambandhat honare ghatak rog vayachya 60 vya varshanantar adhik prachalit aahet.
kahee prakarane varshane midaleli asatat aanee etch. ell. aii. upprakar etch. ell. aii.-dii. aar. 3, etch. ell. aii.-dii. aar. 52 aanee etch. ell. aii.-dii. aar. 6 heey svayampratikar tvachavishodhache lakshan nirman karatat asey disate.
nidan.
dermatomyocytische nidan paach nikashanvar aadharit aahe, jeey polymyocytischya sandarbhat bhinn nidan karnyasathi dekhil vaparale jaatatah
pachva nikash mhanje dermatomyocytis aanee polymyocytismadhye farak karnara; jarr 1 teey 4 paiki teen vastoo 5 vyatirikt asateel, tarr sambhavya 5 vyatirikt konatyahi donsah asateel aanee 5 vyatirikt fakt eka asalyas shakya asel tarr dermatomyocytisasathi nidan nishchit manale jaate.
dermatomyocytis haah svayampratibandhakanshi, visheshtah antinuclear antibodieshee (aii. aine. aii.) sambandhit aahe. dii. emm. chachanimadhye aine. aii. chee chachani positive aalelya sumare 80 takke lokanmadhye aanee sumare 30 takke lokanmadhye myocytis-vishisht svayampratibandh asatat jyat eminosyl-trana syntheteseschya (anty-synthetage antibodies) antibodiescha samavesh asato, jyat hystidine-trana legace (anty-joou1) viruddha antibodies, signal recognition kann (ess. aar. pii.) sathee antibodies aanee anty-emm. aay.-2 antibodiescha samavesh asato.
chumbakeey anunad imaging snayunchya biopsyla margadarshan karnyasathi aanee antargat avyavanchya sahbhagachi tapasani karnyasathi upayukt tharoo shakate; ksh-kiranancha vapar sandhe sahbhag aanee calcification tapasani karnyasathi keela jaau shakato.
jarr keval tvachevar parinam jhaala asel aanee 6 mahinyanpeksha jaast kaal snayuncha kamkuvatpana disun aala nasel tarr dermatomyocytischya dilelya prakaranala amyopathic dermatomyocytis mhanoon vargikrut kele jaau shakate.
vargikaran.
dermatomycytis haah pranaligat sanyojak ootee vikarancha eka prakar aahe, rogancha eka varg jyamadhye anekada svayampratikarshaktiche apkriya samavisht asate.
polymyocytis, necrotising autoimmune myocytis, karkarog-sambandhit myocytis aanee viral samavesh boddy myocytisus, yala idiopathic inflammatory myopathy mhanoon dekhil vargikrut kele gele aahe.
praudhatvapurvi udbhavananya yaa vikaracha eka prakar kishore dermatomyocytis mhanoon olakhala jaato.
upchar.
dermatomyocytisvar konatahi upchar dnyat nahee, parantu lakshananvar upchar kele jaau shakatat. paryayanmadhye aushadhe, sharirik upchar, vyayam, ushma upchar (microwave aanee ultrasounds), orthotics aanee sahayyak upkarane aanee vishranti yancha samavesh aahe. dermatomyocytisasathi pramanit upchar heey eka corticosteroid aushadh aahe, jeey ektar golichya swarupat kinwa antahshira paddhatine dile jaate. ezathioprene aanee methotrexet yasarkhi rogpratikarak shakti qamee karnari aushadhe, jeey lock prednisonla changla pratisad dett naheet, tyanchyat jaljal qamee karoo shakatat. antahshira immunoglobulincha vapar karoon niyatkalik upchar dekhil punarprapti sudharu shakatat. dermatomycytisshy sambandhit dahavar upchar karnyasathi vaparlya jaanarya itar rogpratikarak shakti qamee karananya ghatakanmadhye cyclosporin aii, cyclophosphamide aanee tachrolimus yancha samavesh hoto.
snayunche ksheen hone rokhanyasathi aanee snayunchi takad aanee gatichi shreni parat midvanyasathi samanyatah sharirik upcharpaddhatichi shifaras kelee jaate. dermatomycytis asalelya anek vyaktinna tyanchya tvachechya vikarasathi samyik corticosteroidsarkh samyik malamachi aavashyakta asoo shakate. tyanni uchch-sanrakshn sunscreen aanee snrakshak kapade ghatale pahijet. majjatantu dukhane aanee varanvar sansarg honyas karanibhoot asalelya calciumchya sathya kadhoon taknyasathi shastrakriyechi aavashyakta asoo shakate.
puralanvar upchar karnyasathi maleria virodhi aushadhe, visheshtah hydroxychloroquine aanee kloroquine vaparali jaatat, jase qee samaan paristhitinsathi kele jaate.
jevha lock itar upcharanna pratisad dett naheet tevha retooksimabcha vapar keela jaato.
2016 paryant, praudhanmadhil amyopathic dermatomycytischya upcharanmadhye thos purava navhata; prakashit upcharanmadhye maleria virodhi aushadhe, steroids, tvachevar kinwa tondawate kinwa lagoo kelele, calcinurin avarodhak tvachevar lagoo kelele, dapsone, intravenous immunoglobulin, methotrexet, azathioprene aanee mycophenolate mofetil yancha samavesh hota. yapaiki ekahi phaar prabhavi disat nahee; tyapaiki, antahshira immunoglobulinche sarvottam parinam jhaale aahet.
purvasoochana.
prednisone, intravenous immunoglobulin, plasmapherasis, chemotherapy aanee itar aushadhe yasarkhya aadhunik upcharanchya aagmanapurvi, rognidan kharab hote.
dermatomycytischi tvachevaril lakshane myocytischya sudharanechya samantar upcharpaddhatisaha sudharu shakatat kinwa nasoo shakatat. kahee lokanmadhye, kamkuvatpana aanee pural ekatra sodatat. itaranmadhye, doghancha sambandh nahee, eka kinwa dusaryavar niyantran thevane adhik aavhanatmak aahe. anekada, snayunchya rogavar purese niyantran thevalyanantar tvachecha rog tikoon rahato.
hriday kinwa fuffusanvar parinam jhalyas yaa sthitimule mrutyucha dhoka khoop jaast asato.
mahamarishastra.
dii. emm. chey praman 40-50 vayogatatil sarvadhik aahe, parantu haah rog serve vayogatatil lokanna prabhavit karoo shakato. yacha purushanpeksha striyanvar jaast parinam hoto. dii. emm. chaa pradurbhav darr 100,000 lokanmage eka teey 22 paryant asato.
itihas.
nidanvishayak nikash 1975 madhye prastavit karnyat aale aanee teey mothya pramanat sweekarle gele. amyopathic dm, jyala dm sine myocytis dekhil mhanatat, tyala 2002 madhye naav denyat aale.
sanshodhan.
2016 paryant, dii. emm. chya karananvar tasech biomarkerswar sanshodhan suru hote; dii. emm. madhye khaleel aushadhanchya vaparasathi vaidyakeey chachanya suru hotyah azulemic acid (tappa II), adrenocorticotropic samprerak jail (tappa IV, open label), imo-8400, toll-sarkhya receptor 7,8 aanee 9 (ph II), abetacipt (tappa IV, open label) aanee soodiyam theosulphate (tappa II).