iindaba

Ukusabela kweziyobisi kunye ne-eosinophilia kunye neempawu zenkqubo (DRESS), eyaziwa ngokuba yi-hypersensitivity syndrome ebangelwa ngamachiza, yintsholongwane enzima ye-T-cell-mediated cutaneous reaction ebonakaliswe yi-rash, fever, ukubandakanyeka kwamalungu angaphakathi, kunye neempawu zenkqubo emva kokusetyenziswa ixesha elide kweziyobisi ezithile.
UKUNXABA kwenzeka malunga nesi-1 kwi-1,000 ukuya kwisi-1 kwi-10,000 yezigulane ezifumana iyeza, ngokuxhomekeke kuhlobo lwechiza elibangelayo. Uninzi lweemeko ze-DRESS zibangelwa ngamachiza amahlanu, ngokwehla kolandelelwano lwezehlo: i-allopurinol, i-vancomycin, i-lamotrigine, i-carbamazepine, kunye ne-trimethopridine-sulfamethoxazole. Nangona i-DRESS inqabile ngokwentelekiso, ichaza ukuya kuthi ga kwi-23% yeziphumo zeziyobisi zesikhumba kwizigulane ezibhedlele.Iimpawu zeProdromal ze-DRESS (impendulo yeziyobisi kunye ne-eosinophilia kunye neempawu zenkqubo) ziquka umkhuhlane, i-malaise jikelele, umqala obuhlungu, ubunzima bokugwinya, ukubetha, ukutshisa kwesikhumba, okanye ukudibanisa oku kungasentla. Emva kweli nqanaba, izigulana zihlala zinerhashalala efana nemasisi eqala enkalweni nasebusweni kwaye ngokuthe ngcembe inwenwele, ekugqibeleni igqume ngaphezu kwe-50% yolusu emzimbeni. I-edema yobuso yenye yeempawu ze-DRESS kwaye isenokuthi ibe mandundu okanye ikhokelele kwi-oblique ear lobe crease entsha, enceda ukwahlula I-DRESS kwirhashalala yeziyobisi engantsonkothanga njengemasisi.

Izigulane ezine-DRESS zinokubonisa ngeentlobo ezahlukeneyo zezilonda, ezibandakanya i-urticaria, i-eczema, utshintsho lwe-lichenoid, i-exfoliative dermatitis, i-erythema, izilonda ezijoliswe kuzo, i-purpura, i-blisters, i-pustules, okanye inhlanganisela yezi zinto. Izilonda ezininzi zesikhumba zinokubakho kwisigulane esinye ngexesha elinye okanye zitshintshe njengoko isifo siqhubeka. Kwizigulane ezinolusu olumnyama, i-erythema yokuqala isenokungabonakali, ngoko kufuneka ihlolwe ngononophelo phantsi kweemeko zokukhanya ezilungileyo. Iipustules zixhaphake ebusweni, entanyeni nasesifubeni.

Kuphononongo olulindelekileyo, oluqinisekisiweyo lweRegistry yaseYurophu yeSerious Cutaneous Adverse Reactions (RegiSCAR), i-56% yezigulane ze-DRESS ziye zavelisa ukudumba okuncinci kwe-mucosal kunye nokhukuliseko, kunye ne-15% yezigulane ezinokuvuvukala kwe-mucosal ebandakanya iindawo ezininzi, ngokuqhelekileyo i-oropharynx. yandulela iimpawu zolusu. Irhashalala ngokuqhelekileyo ihlala ngaphezu kweeveki ezimbini kwaye inexesha elide lokubuyisela, xa i-superficial desquamation yeyona nto iphambili. Ukongeza, nangona kunqabile kakhulu, kukho inani elincinci lezigulane ezine-DRESS ezinokungahambi kunye ne-rash okanye i-eosinophilia.

Izilonda zenkqubo ye-DRESS zidla ngokubandakanya igazi, isibindi, izintso, imiphunga, kunye neenkqubo zentliziyo, kodwa phantse yonke inkqubo yamalungu (kuquka i-endocrine, i-gastrointestinal, i-neurological, i-ocular, kunye ne-rheumatic systems) inokubandakanyeka. Kuphononongo lwe-RegiSCAR, i-36 yepesenti yezigulane ubuncinane i-extra-cutaneous ilungu elibandakanyekayo, kwaye i-56 yepesenti inamalungu amabini okanye ngaphezulu abandakanyekayo. I-Atypical lymphocytosis yeyona nto ixhaphakileyo kunye neyokuqala ye-hematological abnormality, ngelixa i-eosinophilia iqhele ukwenzeka kumanqanaba okugqibela esi sifo kwaye inokuqhubeka.
Emva kolusu, isibindi lelona lungu liqinileyo lichaphazelekayo. Amanqanaba e-enzyme yesibindi aphakanyisiweyo angenzeka ngaphambi kokuba kuvele irhashalala, ngokuqhelekileyo ukuya kwinqanaba elincinci, kodwa ngamanye amaxesha anokufikelela kumaxesha angama-10 kumda ophezulu wesiqhelo. Uhlobo oluqhelekileyo lokulimala kwesibindi yi-cholestasis, elandelwa yi-cholestasis edibeneyo kunye nokulimala kwe-hepatocellular. Kwiimeko ezinqabileyo, ukungaphumeleli kwesibindi esibukhali kunokuba nzima ngokwaneleyo ukuba kufuneke ukutshintshwa kwesibindi. Kwiimeko ze-DRESS enokungasebenzi kakuhle kwesibindi, iklasi yeyeza eliqhelekileyo le-pathogenic yi-antibiotics. Uphononongo olucwangcisiweyo luhlalutye izigulane ze-71 (abadala be-67 kunye nabantwana be-4) kunye ne-DRES ehambelana ne-renal sequelae. Nangona uninzi lwezigulana zinomonakalo wesibindi ngaxeshanye, isi-1 kwisigulana esi-5 sikhoyo ngokubandakanyeka kwezintso kuphela. Amayeza okubulala iintsholongwane ayengawona mayeza axhaphakileyo anxulumene nomonakalo wezintso kwizigulane ze-DRESS, kunye ne-vancomycin ebangela i-13 ekhulwini lomonakalo wezintso, ilandelwa yi-allopurinol kunye ne-anticonvulsants. Ukulimala okubukhali kwezintso kubonakaliswe ngokunyuka kwenqanaba le-serum creatinine okanye ukunciphisa izinga lokuhluza i-glomerular, kwaye ezinye iimeko zihamba kunye neproteinuria, i-oliguria, i-hematuria okanye zonke ezintathu. Ukongeza, kusenokubakho kuphela ihematuria okanye iproteinuria, okanye akukho mchamo. I-30% yezigulane ezichaphazelekayo (i-21/71) yafumana unyango lwe-renal replacement, kwaye ngelixa izigulane ezininzi ziphinde zafumana umsebenzi wezintso, kwakungacaci ukuba kukho i-sequelae yexesha elide. Ukubandakanyeka kwemiphunga, okubonakaliswe kukuphefumula okufutshane, ukukhohlela okomileyo, okanye zombini, kuchazwe kwi-32% yezigulane ze-DRESS. Ezona zinto zixhaphakileyo zemiphunga ekuhlolweni kwemifanekiso ziquka ukungena kwangaphakathi, i-acute Respiratory Distress Syndrome kunye ne-pleural effusion. Iingxaki ziquka inyumoniya ebukhali ye-interstitial, inyumoniya ye-lymphocytic interstitial, kunye ne-pleurisy. Ekubeni i-DRESS yemiphunga isoloko ifunyaniswa ngendlela engeyiyo njengenyumoniya, ukuxilongwa kufuna iqondo eliphezulu lokuphaphela. Phantse zonke iimeko zokubandakanyeka kwemiphunga zihamba kunye nolunye ukungasebenzi kakuhle kwamalungu. Kolunye uphononongo olucwangcisiweyo, ukuya kuthi ga kwi-21% yezigulane ze-DRESS zine-myocarditis. I-Myocarditis inokulibaziseka iinyanga emva kokuba ezinye iimpawu ze-DRESS zithobile, okanye ziqhubekile. Iindidi zivela kwi-acute eosinophilic myocarditis (ukuxolelwa kunye nonyango lwe-immunosuppressive lwexesha elifutshane) ukuya kwi-acute necrotizing eosinophilic myocarditis (ukufa okungaphezu kwama-50% kunye nokusinda okuphakathi kweentsuku ezi-3 ukuya kwezi-4 kuphela). Izigulane ezine-myocarditis zihlala zikhona nge-dyspnea, intlungu yesifuba, i-tachycardia, kunye ne-hypotension, ehamba kunye namanqanaba aphezulu e-enzyme ye-myocardial, utshintsho lwe-electrocardiogram, kunye nokungahambi kakuhle kwe-echocardiographic (njenge-pericardial effusion, i-systolic dysfunction, i-ventricular septal hypertrophy, kunye nokungaphumeleli kwe-biventricular). I-cardiac magnetic resonance imaging ingabonisa izilonda ze-endometrial, kodwa ukuxilongwa okuqinisekileyo ngokuqhelekileyo kufuna i-endometrial biopsy. Ukubandakanyeka kwe-Lung kunye ne-myocardial ayiqhelekanga kwi-DRESS, kwaye i-minocycline yenye yezona zinto zixhaphakileyo zokuthobisa.

Inkqubo yokulinganisa i-European RegiSCAR iqinisekisiwe kwaye isetyenziselwa ukuxilongwa kwe-DRESS (Itheyibhile 2). Inkqubo yokulinganisa isekelwe kwiimpawu ezisixhenxe: ukushisa komzimba okungundoqo ngaphezu kwe-38.5 ° C; Ukwandiswa kwe-lymph nodes kwiindawo ezimbini ubuncinane; I-Eosinophilia; I-lymphocytosis engabonakaliyo; IRash (egubungela ngaphezu kwe-50% yendawo yomzimba, iimpawu ze-morphological manifestations, okanye iziphumo ze-histological ezihambelana ne-hypersensitivity yeziyobisi); Ukubandakanyeka kwamalungu angaphandle kwesikhumba; Kwaye uxolelo olude (ngaphezu kweentsuku ezili-15).
Uluhlu lwamanqaku ukusuka ku--4 ukuya kwi-9, kwaye ukuqinisekiswa kokuxilongwa kunokwahlula ngamanqanaba amane: amanqaku angaphantsi kwe-2 abonisa ukuba akukho sifo, i-2 ukuya kwe-3 ibonisa isifo esinokwenzeka, i-4 ukuya kwi-5 ibonisa isifo esinokwenzeka kakhulu, kwaye ngaphezu kwe-5 ibonisa ukuxilongwa kwe-DRESS. Amanqaku e-RegiSCAR abaluleke kakhulu ekuqinisekiseni ukuqinisekiswa kweemeko ezinokwenzeka ngenxa yokuba izigulane zinokuthi zihlangabezane ngokupheleleyo nazo zonke iindlela zokuxilonga ekuqaleni kwesi sifo okanye zingafumananga uvavanyo olupheleleyo oluhambelana namanqaku.

INXABA kufuneka yahlulwe kwezinye iziphumo ezibi zolusu, kubandakanywa i-SJS kunye neziphazamiso ezinxulumene noko, i-toxic epidermal necrolysis (TEN), kunye ne-acute generalized exfoliating impetigo (AGEP) (Figure 1B). Ixesha lokufukamela le-DRESS lidla ngokuba lide kunezinye izenzo ezimandundu zolusu. I-SJS kunye ne-TEN zikhula ngokukhawuleza kwaye zihlala zizisombululo zodwa ngaphakathi kwe-3 ukuya kwiiveki ze-4, ngelixa iimpawu ze-DRESS zivame ukuzingisa. Nangona ukubandakanyeka kwe-mucosal kwizigulane ze-DRESS kunokufuna ukwahlula kwi-SJS okanye kwi-TEN, izilonda zomlomo we-mucosal kwi-DRESS zidla ngokuba zincinci kwaye zincinci ukopha. Uphawu lwe-edema yesikhumba oluphawulweyo lwe-DRESS lunokukhokelela kwi-catatonic blisters kunye nokhukuliseko, ngelixa i-SJS kunye ne-TEN zibonakaliswa ngokukhupha i-epidermal egcweleyo kunye ne-lateral tension, ehlala ibonisa uphawu luka-Nikolsky oluhle. Ngokwahlukileyo, i-AGEP idla ngokubonakala kwiiyure ukuya kwiintsuku emva kokuvezwa kweziyobisi kwaye isombulule ngokukhawuleza kwiiveki ezi-1 ukuya kwezi-2. I-rash ye-AGEP igobile kwaye yenziwe ngama-pustules aqhelekileyo angagcinwanga kwiifollicle zeenwele, ezihluke ngandlel' ithile kwiimpawu ze-DRESS.
Uphononongo olulindelekileyo lubonise ukuba i-6.8% yezigulane ze-DRESS zineempawu zazo zombini i-SJS, i-TEN okanye i-AGEP, apho i-2.5% ithathwa njenge-overlapping enzima kakhulu ye-skin reactions. Ukusetyenziswa kwemigaqo yokuqinisekisa i-RegiSCAR kunceda ukuchonga ngokuchanekileyo le miqathango.
Ukongezelela, i-rashes yeziyobisi eziqhelekileyo ezifana nemasisi zivame ukuvela kwiiveki ezi-1 ukuya kwezi-2 emva kokuvezwa kweyeza (ukutshatyalaliswa kwakhona ngokukhawuleza), kodwa ngokungafaniyo ne-DRESS, ezi ngqungquthela aziqhelekanga ukuhamba ne-transaminase ephakamileyo, ukunyuka kwe-eosinophilia, okanye ixesha elide lokubuyisela kwiimpawu. INXABA nayo ifuna ukwahlulwa kwezinye iindawo zezifo, kuquka i-hemophagocytic lymphohistiocytosis, i-vascular immunoblastic T-cell lymphoma, kunye nesifo esibukhali sokuxhunyelelwa-kulwa ne-host.

Imvumelwano yeengcali okanye izikhokelo malunga nonyango lwe-DRESS azikaphuhliswa; Iingcebiso zonyango ezikhoyo zisekelwe kwidatha yokujonga kunye noluvo lwengcali. Izifundo zokuthelekisa ukukhokela unyango nazo zinqongophele, ngoko ke iindlela zonyango azifani.
Sula unyango lweyeza elibangela isifo
Inyathelo lokuqala nelona libalulekileyo kwi-DRESS kukuchonga kunye nokuyeka elona chiza linokuba ngunobangela. Ukuqulunqa iitshathi ezineenkcukacha zamayeza kwizigulana kunokunceda kule nkqubo. Ngokwenza itshathi yeziyobisi, oogqirha banokubhala ngokucwangcisiweyo onke amachiza anokuthi abangele izifo kwaye bahlalutye ubudlelwane bexeshana phakathi kokuvezwa kweziyobisi kunye nerhashalala, i-eosinophilia, kunye nokubandakanyeka kwamalungu. Besebenzisa olu lwazi, oogqirha banokulihlola iyeza elona linokubangela i-DRESS kwaye bayeke ukusebenzisa elo chiza kwangethuba. Ukongeza, oogqirha banokubhekisa kwii-algorithms ezisetyenziselwa ukumisela unobangela weziyobisi kwezinye iziphumo ezibi zolusu.

Amayeza - glucocorticoids
I-Systemic glucocorticoids yeyona ndlela iphambili yokubangela uxolelo lwe-DRESS kunye nokunyanga ukuphindaphinda. Nangona idosi yokuqala yesiqhelo yi-0.5 ukuya kwi-1 mg/d/kg ngosuku (ilinganiswe ngokulinganayo ne-prednisone), kukho ukusilela kwezilingo zeklinikhi ezivavanya ukusebenza kwe-corticosteroids ye-DRESS, kunye nezifundo kwiidosi ezahlukeneyo kunye nerejimeni zonyango. Umthamo we-glucocorticoids akufanele uncitshiswe ngokungenamkhethe de kubonwe ukuphuculwa okucacileyo kweklinikhi, njengokunciphisa i-rash, i-eosinophil penia, kunye nokubuyiselwa komsebenzi womzimba. Ukunciphisa umngcipheko wokuphindaphinda, kucetyiswa ukuba unciphise ngokuthe ngcembe umthamo we-glucocorticoids ngaphezu kwe-6 kwiiveki ze-12. Ukuba umthamo oqhelekileyo awusebenzi, ngoko "i-shock" yonyango ye-glucocorticoid, i-250 mg imihla ngemihla (okanye ilingana) kwiintsuku ze-3, inokuqwalaselwa, ilandelwa ukunciphisa ngokuthe ngcembe.
Kwizigulane ezine-DRESS ephakathi, i-topical corticosteroids esebenzayo kakhulu inokuba lukhetho olusebenzayo lonyango. Ngokomzekelo, u-Uhara et al. ingxelo yokuba izigulane ezili-10 ze-DRESS zachacha ngempumelelo ngaphandle kwe-systemic glucocorticoids. Nangona kunjalo, ngenxa yokuba akucaci ukuba zeziphi izigulana ezinokuphepha ngokukhuselekileyo unyango lwenkqubo, ukusetyenziswa ngokubanzi kwezonyango zonyango akukhuthazwa njengenye indlela.

Gwema unyango lweglucocorticoid kunye nonyango olujoliswe kuyo
Kwizigulane ze-DRESS, ngakumbi ezo zisengozini enkulu yeengxaki (ezifana nokusuleleka) ekusebenziseni imilinganiselo ephezulu ye-corticosteroids, unyango lokuphepha lwe-corticosteroid lunokuqwalaselwa. Nangona kukho iingxelo zokuba i-immunoglobulin ye-intravenous (IVIG) inokusebenza kwezinye iimeko, uphando oluvulekileyo lubonise ukuba unyango lunomngcipheko omkhulu wemiphumo emibi, ngakumbi i-thromboembolism, ekhokelela izigulane ezininzi ekugqibeleni zitshintshele kwi-systemic glucocorticoid therapy. Ukusebenza okunokwenzeka kwe-IVIG kunokunxulumana nefuthe layo lokususa i-antibody, enceda ukuthintela usulelo lwentsholongwane okanye ukuphinda kusebenze intsholongwane. Nangona kunjalo, ngenxa yeedosi ezinkulu ze-IVIG, ayinakulungela izigulana ezinokusilela kwentliziyo, ukungaphumeleli kwezintso, okanye ukungaphumeleli kwesibindi.
Ezinye iindlela zonyango ziquka i-mycophenolate, i-cyclosporin kunye ne-cyclophosphamide. Ngokuthintela ukusebenza kweeseli ze-T, i-cyclosporine ivimba i-gene transcription ye-cytokines efana ne-interleukin-5, ngaloo ndlela inciphisa ukuqeshwa kwe-eosinophilic kunye ne-cell-specific T cell activation. Uphononongo olubandakanya izigulane ezihlanu eziphathwe nge-cyclosporine kunye nezigulane ze-21 eziphathwe nge-systemic glucocorticoids zibonise ukuba ukusetyenziswa kwe-cyclosporine kwakudityaniswa namazinga aphantsi okuqhubela phambili kwesifo, ukuphuculwa kwemilinganiselo yeklinikhi kunye nelebhu, kunye nokuhlala okufutshane esibhedlele. Nangona kunjalo, i-cyclosporine okwangoku ayithathwa njengonyango lodidi lokuqala lwe-DRESS. I-Azathioprine kunye ne-mycophenolate zisetyenziselwa ukugcinwa konyango kunonyango lwe-induction.
Izilwa-buhlungu ze-Monoclonal zisetyenziselwe ukunyanga i-DRESS. Ezi ziquka i-Mepolizumab, i-Ralizumab, kunye ne-benazumab evimba i-interleukin-5 kunye ne-receptor axis yayo, i-Janus kinase inhibitors (efana ne-tofacitinib), kunye ne-anti-CD20 monoclonal antibodies (efana ne-rituximab). Phakathi kwezi zonyango, i-anti-interleukin-5 iziyobisi zibhekwa njengezona zifikeleleke, ezisebenzayo kunye nonyango olukhuselekileyo lwe-induction. Indlela yokusebenza inokuthi ihambelane nokuphakama kwangaphambili kwamanqanaba e-interleukin-5 kwi-DRESS, edla ngokunyanzeliswa ziiseli ze-T zeziyobisi. I-Interleukin-5 ngumlawuli oyintloko we-eosinophils kwaye ixanduva lokukhula kwabo, ukuhlukana, ukuqeshwa, ukusebenza kunye nokuphila. Amachiza e-Anti-interleukin-5 asetyenziswa ngokuqhelekileyo ukunyanga izigulane ezisenayo i-eosinophilia okanye ukungasebenzi kakuhle kwamalungu emva kokusetyenziswa kwe-systemic glucocorticoids.

Ubude bexesha lonyango
Unyango lwe-DRESS kufuneka lube lolwakho kakhulu kwaye luhlengahlengiswe ngokokuqhubekeka kwesifo kunye nempendulo yonyango. Izigulana ezine-DRESS zifuna ukulaliswa esibhedlele, kwaye malunga nekota yezi meko zifuna ulawulo lokhathalelo olunzulu. Ngethuba lokulaliswa esibhedlele, iimpawu zesigulane zivavanywa imihla ngemihla, uvavanyo olubanzi lomzimba lwenziwa, kwaye izibonakaliso zebhubhoratri zihlala zijongwa ukuvavanya ukubandakanyeka kwamalungu kunye noshintsho kwi-eosinophils.
Emva kokukhutshwa, ukuvavanywa kokulandelelana kweveki kusafuneka ukujonga utshintsho kwimeko kunye nokulungelelanisa isicwangciso sonyango ngexesha. Ukuphindaphinda kunokuvela ngokuzenzekelayo ngexesha lokuncipha kwedosi yeglucocorticoid okanye emva kokuxolelwa, kwaye kunokubonisa njengempawu enye okanye isilonda selungu lendawo, ngoko ke izigulane kufuneka zibekwe esweni ixesha elide kwaye ngokubanzi.