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Jawaabta daroogada ee eosinophilia iyo calaamadaha nidaamka (DRESS), oo sidoo kale loo yaqaan 'hypersensitivity syndrome' ee daroogada ay keentay, waa falcelin xun oo xun oo T-cell dhexdhexaad ah oo lagu garto finan, qandho, ku lug lahaanshaha xubnaha gudaha, iyo calaamadaha nidaamka ka dib isticmaalka dheeraadka ah ee daawooyinka qaarkood.
Labbisku waxa uu ku dhacaa ku dhawaad ​​1 1,000 ilaa 1 10,000 bukaan ah ee qaata dawooyinka, taas oo ku xidhan nooca dawada kicinaysa. Inta badan kiisaska DRESS waxaa sababay shan dawo, oo hoos u dhacay sida ay u dhaceen: allopurinol, vancomycin, lamotrigine, carbamazepine, iyo trimethopridine-sulfamethoxazole. Inkasta oo DRESS uu yahay mid naadir ah, waxay ka dhigan tahay ilaa 23% falcelinta daroogada maqaarka ee bukaannada isbitaallada. Calaamadaha Prodromal ee DRESS (ka jawaab celinta daroogada ee eosinophilia iyo calaamadaha habdhiska) waxaa ka mid ah qandho, jirro guud, cune xanuun, liqitaan adag, cuncun, maqaarka gubanaya, ama isku darka korka. Marxaladdan ka dib, bukaanku waxay inta badan ku dhacaan finan u eg jadeecada oo ka soo bilaabma jilibka iyo wejiga oo si tartiib ah u faafa, ugu dambeyntiina daboolaya in ka badan 50% maqaarka jirka. Bararka wejiga waa mid ka mid ah astaamaha lagu garto DRESS waxaana laga yaabaa inay sii xumeeyaan ama u horseedaan xinjir dhegta oo cusub, taasoo ka caawisa in la kala saaro dhaymada iyo finan jadeecada oo kale ah oo aan dhib lahayn.

Bukaanka qaba DRESS waxaa laga yaabaa inay soo bandhigaan nabaro kala duwan, oo ay ku jiraan urtikaria, canbaarta, isbeddellada lichenoid, maqaarka maqaarka ah, erythema, nabarrada qaabka bartilmaameedka ah, purpura, finan, finan, ama kuwan la isku daray. Nabaro badan oo maqaarka ah ayaa laga yaabaa inay isla bukaan isku mar ku jiraan ama isbeddelaan marka uu cudurku sii socdo. Bukaannada leh maqaarka madow, erythema hore lagama yaabo in la dareemo, sidaas darteed waxay u baahan tahay in si taxadar leh loo baaro xaaladaha iftiinka wanaagsan. Malaxyadu waxay ku badan yihiin wejiga, qoorta iyo aagga laabta.

Diiwaangelinta Yurub ee la ansixiyay ee falcelinta xun xun ee halista ah (RegiSCAR), 56% bukaannada DRESS waxay sameeyeen caabuqa xuubka khafiifka ah iyo nabaad guurka, iyadoo 15% bukaannada qaba caabuq xuubka xabka ah oo ku lug leh goobo badan, badiyaa oropharynx. balaadhinta xitaa waxay ka horraysa calaamadaha maqaarka. Finanku wuxuu caadi ahaan qaataa wax ka badan laba toddobaad waxayna leeyihiin muddo dheer oo soo kabasho ah, marka hoos u dhac ku yimaadda ay tahay qaabka ugu muhiimsan. Intaa waxaa dheer, inkasta oo aad dhif u ah, waxaa jira tiro yar oo bukaanno ah oo qaba DRESS kuwaas oo laga yaabo inaysan la socon finan ama eosinophilia.

Nabarada habaysan ee DRESS waxay badanaa ku lug leeyihiin dhiiga, beerka, kelyaha, sambabada, iyo hababka wadnaha, laakiin ku dhawaad ​​nidaamka xubnaha (oo ay ku jiraan endocrine, caloosha, neerfaha, indhaha, iyo hababka rheumatic) ayaa ku lug yeelan kara. Daraasadda RegiSCAR, boqolkiiba 36 bukaannada ayaa lahaa ugu yaraan hal xubin oo ka baxsan maqaarka oo ku lug leh, iyo 56 boqolkiiba waxay lahaayeen laba ama in ka badan oo xubno ah oo ku lug leh. Atypical lymphocytosis waa cilladda ugu badan iyo tan ugu horreysa ee aan caadiga ahayn, halka eosinophilia ay badanaa ku dhacdo marxaladaha dambe ee cudurka oo laga yaabo inay sii jirto.
Maqaarka ka dib, beerku waa xubinta adag ee inta badan saameeya. Heerarka enzymes beerka ee sarreeya ayaa laga yaabaa inay dhacaan ka hor inta aysan finanku soo bixin, badiyaa ilaa heer khafiif ah, laakiin marmar waxay gaari karaan ilaa 10 jeer xadka sare ee caadiga ah. Nooca ugu badan ee dhaawaca beerka waa cholestasis, oo ay ku xigto cholestasis isku dhafan iyo dhaawaca hepatocellular. Xaalado naadir ah, beer-xumada degdega ahi waxay noqon kartaa mid aad u daran oo u baahan beerka beddelka. Xaaladaha DRESS ee leh cillad beerka, fasalka ugu badan ee cudurka keena waa antibiyootiga. Dib-u-eegis habaysan ayaa lagu falanqeeyay 71 bukaan (67 qaangaar ah iyo 4 carruur ah) oo leh daba-galka kelyaha ee la xiriira DRES. In kasta oo bukaannada intooda badan ay leeyihiin dhaawac beerka ah, 5tii bukaanba 1 ayaa la soo baxay ka qaybqaadashada kelyaha oo keliya. Antibiyootiga ayaa ah daawooyinka ugu badan ee lala xiriiriyo dhaawaca kelyaha ee bukaanka DRESS, iyadoo vancomycin uu sababay 13 boqolkiiba dhaawaca kelyaha, oo ay ku xigto allopurinol iyo anticonvulsans. Dhaawaca kelyaha ee ba'an waxaa lagu gartaa heerka serum creatinine oo kordhay ama hoos u dhaca heerka sifaynta glomerular, iyo xaaladaha qaarkood waxaa la socday proteinuria, oliguria, hematuria ama dhammaan saddexda. Intaa waxaa dheer, waxaa jiri kara oo kaliya hematuria ama proteinuria, ama xitaa kaadi ma jirto. 30% bukaannada ay saamaysay (21/71) waxay heleen daawayn beddelka kelyaha, halka bukaanno badani ay dib u heleen shaqadii kelyaha, ma cadda in ay jiraan daba-gal muddo dheer ah. Ku lug lahaanshaha sambabada, oo lagu garto neefta oo gaaban, qufac qalalan, ama labadaba, ayaa lagu soo warramey 32% bukaanada DRESS. Waxyeellada sambabada ee ugu badan ee baaritaanka sawir-qaadista waxaa ka mid ah galitaanka interstitial, xanuunka neef-mareenka oo ba'an iyo dhiigbax. Dhibaatooyinka waxaa ka mid ah oof wareenka interstitial oo ba'an, oof wareenka interstitial lymphocytic, iyo pleurisy. Maadaama DRESS sambabada inta badan si khaldan loo ogaado sambabada, ogaanshaha waxa uu u baahan yahay feejignaan sare. Ku dhawaad ​​dhammaan kiisaska ku lug lahaanshaha sambabada waxaa weheliya hawl-gabka xubnaha kale ee adag. Dib u eegis kale oo habaysan, ilaa 21% bukaanada DRESS waxa ku dhacay myocarditis. Myocarditis waxaa laga yaabaa inuu dib u dhigo bilo ka dib marka calaamadaha kale ee DRESS ay hoos u dhacaan, ama xitaa ay sii jiraan. Noocyada waxay u dhexeeyaan eosinophilic myocarditis ba'an (ka-cafiska leh daawaynta difaaca jirka ee muddada-gaaban) ilaa necrotizing eosinophilic myocarditis (dhimashada in ka badan 50% iyo badbaadada dhexe ee 3 ilaa 4 maalmood oo keliya). Bukaanka qaba myocarditis waxay inta badan la kulmaan dyspnea, xanuunka laabta, tachycardia, iyo hypotension, oo ay la socdaan heerarka enzyme myocardial myocardial oo sarreeya, isbeddellada elektiroonigga ah, iyo cilladaha echocardiographic (sida xuubka wadnaha, cilladda systolic, hypertrophy ventricular septal hypertrophy, iyo biventricular failure). Sawirka resonance ee wadnaha ee magnetic wuxuu muujin karaa nabarrada endometrial, laakiin ogaanshaha saxda ah wuxuu caadi ahaan u baahan yahay biopsy endometrial. Ku lug lahaanshaha sambabada iyo wadnaha myocardial aad ayey ugu yar tahay dhaymada, iyo minocycline waa mid ka mid ah wakiilada kicinta ugu badan.

Nidaamka dhibcaha Diiwaangelinta Yurub waa la ansaxiyay waxaana si weyn loogu isticmaalaa ogaanshaha DRESS (Shaxda 2). Nidaamka dhibcaha wuxuu ku salaysan yahay todoba astaamood: heerkulka jidhka xudunta u ah oo ka sarreeya 38.5 ° C; Lafaha qanjidhada oo weyn ugu yaraan laba goobood; Eosinophilia; lymphocytosis aan caadi ahayn; Finanka (oo daboolaya in ka badan 50% aagga dusha sare ee jirka, muuqaalada qaab-dhismeedka dabeecadda, ama natiijooyinka taariikhiga ah ee la socda dareenka xasaasiga ah ee daroogada); Ku lug lahaanshaha xubnaha ka baxsan maqaarka; Iyo cafis dabadheeraad ah (in ka badan 15 maalmood).
Dhibcuhu wuxuu u dhexeeyaa -4 ilaa 9, hubaasha ogaanshaha waxa loo qaybin karaa afar heer: dhibcaha 2 ka hooseeya waxay muujinayaan cudur, 2 ilaa 3 waxay muujinayaan cudur suurtagal ah, 4 ilaa 5 waxay muujinayaan cudur aad u badan, iyo in ka badan 5 waxay muujinayaan ogaanshaha DRESS. Dhibcaha RegiSCAR ayaa si gaar ah faa'iido u leh xaqiijinta dib-u-eegista ee kiisaska suurtagalka ah sababtoo ah bukaannada waxaa laga yaabaa inaysan si buuxda u buuxin dhammaan shuruudaha ogaanshaha horaantii cudurka ama aysan helin qiimeyn dhamaystiran oo la xiriirta dhibcaha.

DRESS waxay u baahan tahay in laga sooco fal-celin xun oo maqaarka kale oo halis ah, oo ay ku jiraan SJS iyo xanuunada la xidhiidha, epidermal necrolysis sun ah (TEN), iyo ba'an guud ahaan exfoliating impetigo (AGEP) (Jaantuska 1B). Muddada ku-soo-baxa ee DRESS caadi ahaan way ka dheer tahay fal-celinta kale ee xun ee maqaarka. SJS iyo TEN waxay u koraan si dhakhso ah waxayna caadi ahaan iskood u xalliyaan 3 ilaa 4 toddobaad gudahood, halka calaamadaha DRESS ay u muuqdaan kuwo sii socda. Inkasta oo ku lug lahaanshaha xuubka xuubka ee bukaanka DRESS laga yaabo in loo baahdo in laga sooco SJS ama TEN, nabarrada xuubka afka ee DRESS caadi ahaan waa khafiif oo dhiig-bax yar. Bararka maqaarka ee calaamadeysan ee DRESS waxay u horseedi kartaa nabaad-guurka labaad ee catatonic iyo nabaad-guur, halka SJS iyo TEN lagu garto lakabka lakabka buuxa ee maqaarka oo leh xiisad dhinaca dambe ah, oo inta badan muujisa calaamadda Nikolsky ee togan. Taas bedelkeeda, AGEP waxay badanaa u muuqataa saacado ilaa maalmo ka dib soo-gaadhista daawada waxayna si degdeg ah u xallisaa 1 ilaa 2 toddobaad gudahood. Finanka AGEP waa qalloocan oo ka kooban malaxyo guud oo aan ku ekayn timaha timaha, taas oo xoogaa ka duwan sifada lebbiska.
Daraasad la filayo ayaa muujisay in 6.8% bukaannada DRESS ay leeyihiin sifooyin labadaba SJS, TEN ama AGEP, kuwaas oo 2.5% loo tixgeliyey inay leeyihiin dareen-celin xun oo maqaarka ah. Isticmaalka shuruudaha ansaxinta RegiSCAR waxay caawisaa in si sax ah loo aqoonsado xaaladahan.
Intaa waxaa dheer, finanka daawada jadeecada ee caadiga ah waxay badanaa soo baxaan 1 ilaa 2 toddobaad gudahood ka dib soo-gaadhista daawada (dib-u-soo-bandhigidda waa dhakhso badan tahay), laakiin si ka duwan DRESS, finankani badanaa lama socdaan transaminase sare, eosinophilia oo kordha, ama waqti dheer oo ka soo kabasho calaamadaha. Labbiska waxa kale oo uu u baahan yahay in laga sooco meelaha kale ee cudurrada, oo ay ku jiraan hemophagocytic lymphohistiocytosis, vascular immunoblastic T-cell lymphoma, iyo cudur tallaal-ka-horjeeda oo degdeg ah.

Fikradda khubarada ama tilmaamaha ku saabsan daaweynta DRESS lama samayn; Talooyinka daawaynta ee jira waxay ku salaysan yihiin xogta fiirsashada iyo ra'yiga khabiirada. Daraasado isbarbardhig ah oo lagu hagayo daawaynta ayaa sidoo kale ka maqan, markaa hababka daawaynta maaha mid isku mid ah.
Daaweyn cad oo cudur keena
Tallaabada ugu horreysa uguna daran ee DRESS waa in la aqoonsado oo la joojiyo daawada ugu badan ee sababa. Samaynta jaantusyo daawo oo faahfaahsan oo loogu talagalay bukaanada ayaa laga yaabaa inay ka caawiso habkan. Marka la eego jaantuska daroogada, takhaatiirta caafimaadku waxay si nidaamsan u diiwaangelin karaan dhammaan daawooyinka cudur-sidaha ee suurtogalka ah waxayna falanqeyn karaan xiriirka ku meel gaarka ah ee ka dhexeeya soo-gaadhista daroogada iyo finan, eosinophilia, iyo ka-qaybgalka xubnaha. Isticmaalka macluumaadkan, takhaatiirtu waxay baari karaan daawada ay u badan tahay inay kiciso DRESS oo ay joojiyaan isticmaalka daawadaas waqtiga. Intaa waxaa dheer, dhakhaatiirtu waxay sidoo kale tixraaci karaan algorithms loo isticmaalo si loo go'aamiyo sababaha daroogada ee falcelinta kale ee xun ee maqaarka.

Daawooyinka - glucocorticoids
Glucocorticoids habaysan ayaa ah hababka asaasiga ah ee keena cafiska DRESS iyo daaweynta soo noqoshada. Inkasta oo qiyaasta bilawga ah ee caadiga ah ay tahay 0.5 ilaa 1 mg / d / kg maalintii (oo lagu qiyaaso prednisone u dhiganta), waxaa jira la'aanta tijaabooyinka kiliinikada ee qiimeynaya waxtarka corticosteroids ee DRESS, iyo sidoo kale daraasado ku saabsan qiyaaso kala duwan iyo hababka daaweynta. Qiyaasta glucocorticoids waa in aan si sabab la'aan ah loo dhimin ilaa laga helayo hagaajin caafimaad oo cad, sida hoos u dhaca finanka, penia eosinophils, iyo soo celinta shaqada xubnaha. Si loo yareeyo khatarta soo noqoshada, waxaa lagu talinayaa in si tartiib tartiib ah loo yareeyo qiyaasta glucocorticoids 6 ilaa 12 toddobaad. Haddii qiyaasta caadiga ah aysan shaqeynin, markaa "naxdin" daaweynta glucocorticoid, 250 mg maalin kasta (ama u dhiganta) 3 maalmood, ayaa la tixgelin karaa, oo ay ku xigto si tartiib tartiib ah.
Bukaannada qaba DRESS khafiif ah, corticosteroids-ka jirka la mariyo oo aad waxtar u leh ayaa laga yaabaa inay noqoto ikhtiyaar daawaynta waxtarka leh. Tusaale ahaan, Uhara et al. ayaa sheegay in 10 bukaan oo DRESS ah ay si guul leh uga soo kabteen iyaga oo aan lahayn glucocorticoids habaysan. Si kastaba ha ahaatee, sababtoo ah ma cadda bukaanada si badbaado leh uga fogaan kara daaweynta nidaamka, isticmaalka baahsan ee daaweynta jirka laguma talinayo beddelka.

Ka fogow daaweynta glucocorticoid iyo daawaynta la beegsanayo
Bukaanjiifka DRESS, gaar ahaan kuwa khatarta sare ugu jira dhibaatooyinka (sida caabuqyada) ee isticmaalka qiyaaso badan oo corticosteroids ah, daawaynta ka fogaanshaha corticosteroid ayaa laga yaabaa in la tixgeliyo. Inkasta oo ay jiraan warbixino sheegaya in immunoglobulin xididka (IVIG) laga yaabo inuu waxtar u yeesho xaaladaha qaarkood, daraasad furan ayaa muujisay in daawadu ay leedahay khatar sare oo saameyn xun ah, gaar ahaan thromboembolism, taasoo keentay bukaanno badan si ay ugu dambeyntii u beddelaan daaweynta glucocorticoid ee nidaamka. Waxtarka suurtagalka ah ee IVIG ayaa laga yaabaa inay la xiriirto saameynteeda nadiifinta antibody-ka, taas oo gacan ka geysaneysa joojinta caabuqa fayraska ama dib-u-kicinta fayraska. Si kastaba ha noqotee, sababtoo ah qiyaaso badan oo IVIG ah, waxaa laga yaabaa inaanay ku haboonayn bukaanada qaba xanuunka wadnaha, kelyaha, ama beerka.
Ikhtiyaarada kale ee daaweynta waxaa ka mid ah mycophenolate, cyclosporin iyo cyclophosphamide. Iyadoo la joojinayo firfircoonida unugyada T, cyclosporine waxay xannibaysaa qoraalka hidda-wadaha ee cytokines sida interleukin-5, taas oo yaraynaysa qoritaanka eosinophilic iyo firfircoonida unugyada T-ga ee daroogada. Daraasad ku lug leh shan bukaan oo lagu daaweeyay cyclosporine iyo 21 bukaan oo lagu daaweeyay nidaamka glucocorticoids ayaa muujisay in isticmaalka cyclosporine ay la xiriirto heerarka hoose ee horumarinta cudurrada, hagaajinta caafimaadka iyo shaybaarka, iyo joogitaanka isbitaalka gaaban. Si kastaba ha ahaatee, cyclosporine hadda looma tixgeliyo daawaynta safka koowaad ee DRESS. Azthioprine iyo mycophenolate ayaa inta badan loo isticmaalaa daawaynta dayactirka halkii ay ka ahaan lahaayeen daawaynta induction.
Unugyada difaaca jirka ee monoclonal ayaa loo isticmaalay daawaynta DRESS. Kuwaas waxaa ka mid ah Mepolizumab, Ralizumab, iyo benazumab kuwaas oo xannibaya interleukin-5 iyo dhidibka soo-dhoweynta, Janus kinase inhibitors (sida tofacitinib), iyo anti-CD20 monoclonal antibodies (sida rituximab). Ka mid ah daawayntan, daawooyinka anti-interleukin-5 ayaa loo arkaa inay yihiin kuwa la heli karo, wax ku ool ah oo badbaado leh daawaynta kicinta. Habka waxtarka leh ayaa laga yaabaa inuu la xiriiro kor u kaca hore ee heerarka interleukin-5 ee DRESS, kaas oo inta badan ay kiciyaan unugyada T-gaar ah ee daroogada. Interleukin-5 waa maamulaha ugu weyn ee eosinophils waxayna mas'uul ka tahay korriintooda, kala duwanaanshahooda, qoritaanka, firfircoonida iyo badbaadada. Daawooyinka Anti-interleukin-5 ayaa caadi ahaan loo isticmaalaa in lagu daweeyo bukaanada weli qaba eosinophilia ama cilladda xubnaha ka dib isticmaalka nidaamka glucocorticoids.

Muddada daaweynta
Daawaynta DRESS waxay u baahan tahay in si heer sare ah loo shakhsiyeeyo oo si firfircoon loo hagaajiyo iyadoo loo eegayo horumarka cudurka iyo jawaabta daawaynta. Bukaanka qaba DRESS caadi ahaan waxay u baahan yihiin isbitaal, iyo afar meelood meel kiisaskan waxay u baahan yihiin maarayn daryeel degdeg ah. Inta lagu jiro isbitaalka, calaamadaha bukaanka ayaa la qiimeeyaa maalin kasta, baaritaan jireed oo dhamaystiran ayaa la sameeyaa, tilmaamayaasha shaybaarka si joogto ah ayaa loola socdaa si loo qiimeeyo ku lug lahaanshaha xubnaha iyo isbeddelada eosinophils.
Ka dib markii la sii daayo, qiimeyn dabagal toddobaadle ah ayaa weli loo baahan yahay si loola socdo isbeddelka xaaladda oo lagu hagaajiyo qorshaha daaweynta wakhtiga. Soo noqoshada ayaa laga yaabaa inay si kedis ah u dhacdo inta lagu jiro hoos u dhaca qiyaasta glucocorticoid ama ka dib marka la cafiyo, waxayna u muuqan kartaa hal calaamad ama dhaawac xubin maxalli ah, markaa bukaanada waxay u baahan yihiin in lala socdo muddo dheer iyo si buuxda.