amakuru

Igisubizo cyibiyobyabwenge hamwe na eosinofilia nibimenyetso bya sisitemu (DRESS), bizwi kandi nka syndrome de hypersensitivite iterwa nibiyobyabwenge, ni T-selile-medrated mediane cutane reaction reaction irangwa no guhubuka, umuriro, uruhare rwimbere rwimbere, nibimenyetso bya sisitemu nyuma yo gukoresha imiti myinshi.
IMYAMBARO iboneka hafi 1 kuri 1.000 kugeza 1 ku barwayi 10,000 bahabwa imiti, bitewe n'ubwoko bw'imiti itera. Umubare munini w’imyambarire yatewe n’ibiyobyabwenge bitanu, uko byagabanutse bikurikirana: allopurinol, vancomycine, lamotrigine, karbamazepine, na trimethopridine-sulfamethoxazole. Nubwo IMYAMBARO idakunze kubaho, ibarirwa kuri 23% yibiyobyabwenge byuruhu kubarwayi bari mubitaro.Ibimenyetso byerekana ibimenyetso byUMWAMBARO (igisubizo cyibiyobyabwenge hamwe na eosinofilia nibimenyetso bya sisitemu) harimo umuriro, kurwara muri rusange, kubabara mu muhogo, ingorane zo kumira, kwishongora, gutwika uruhu, cyangwa guhuza ibivuzwe haruguru. Nyuma yiki cyiciro, abarwayi bakunze kurwara igisebe kimeze nkigisebe gitangirira kumubiri no mumaso bikagenda bikwirakwira buhoro buhoro, amaherezo bitwikiriye uruhu rurenga 50% kumubiri. Indwara yo mu maso ni kimwe mu biranga UMWAMBARO kandi irashobora kwiyongera cyangwa kuganisha ku gutwi gushya kwamatwi matwi, bifasha gutandukanya IMYAMBARO n’indwara idasanzwe iterwa n’ibiyobyabwenge.

Abarwayi bafite IMYAMBARO barashobora kwerekana ibikomere bitandukanye, harimo urticaria, eczema, impinduka za lichenoide, dermatite ya exfoliative, erythema, ibikomere bimeze nkintego, purpura, ibisebe, pustules, cyangwa guhuza ibi. Indwara nyinshi zuruhu zirashobora kuboneka kumurwayi umwe icyarimwe cyangwa guhinduka uko indwara igenda ikura. Ku barwayi bafite uruhu rwijimye, erythma hakiri kare ntishobora kugaragara, bityo rero igomba gusuzumwa neza mugihe cyiza kimurika. Ibibyimba bikunze kugaragara mumaso, ijosi no mugituza.

Mu bushakashatsi buteganijwe, bwemewe bw’Uburayi bw’ubushakashatsi bukomeye bwa Cutaneous Adverse Reaction (RegiSCAR), 56% by’abarwayi ba DRESS barwaye indwara ya mucosal yoroheje ndetse n’isuri, aho 15% by’abarwayi bafite uburibwe bwitwa mucosal burimo imbuga nyinshi, cyane cyane oropharynx.Mu bushakashatsi bwa RegiSCAR, benshi mu barwayi ba DRESS bafite ubwiyongere bukabije bwa lymph node, ndetse no mu barwayi bamwe na bamwe bafite indwara ya lymph node. Ubusanzwe ibisebe bimara ibyumweru birenga bibiri kandi bifite igihe kirekire cyo gukira, mugihe desquamation yo hejuru aribintu nyamukuru. Mubyongeyeho, nubwo ari gake cyane, hariho umubare muto wabarwayi bafite DRESS badashobora guherekezwa no guhubuka cyangwa eosinofilia.

Indwara ya sisitemu yimyambarire isanzwe irimo amaraso, umwijima, impyiko, ibihaha, na sisitemu yumutima, ariko hafi ya buri ngingo yingingo zose (harimo na endocrine, gastrointestinal, neurologiya, ocular, na rheumatique) zirashobora kubigiramo uruhare. Mu bushakashatsi bwa RegiSCAR, 36 ku ijana by'abarwayi bagize byibuze urugingo rumwe rudasanzwe, naho 56 ku ijana babigizemo uruhare ebyiri cyangwa nyinshi. Lymphocytose idasanzwe ni yo ikunze kugaragara kandi idasanzwe ya hematologiya idasanzwe, mu gihe eosinofilia ikunze kugaragara mu bihe byanyuma by’indwara kandi ishobora gukomeza.
Nyuma yuruhu, umwijima ningingo zikunze kwibasirwa ningingo zikomeye. Urwego rwinshi rwimisemburo yumwijima rushobora kubaho mbere yuko igisebe kigaragara, mubisanzwe kurwego rworoheje, ariko rimwe na rimwe birashobora kugera ku nshuro 10 kurenza urugero rusanzwe. Ubwoko bw'imvune zikunze kugaragara ni cholestasis, hagakurikiraho cholestasis ivanze no gukomeretsa hepatocellular. Mubihe bidasanzwe, kunanirwa kwumwijima birashobora kuba bikomeye kuburyo bisaba guhindurwa umwijima. Mugihe cyimyambarire idakora neza yumwijima, icyiciro cyibiyobyabwenge gikunze gutera ni antibiotique. Isuzuma rifatika ryasesenguye abarwayi 71 (abantu bakuru 67 n’abana 4) bafite impyiko zifitanye isano na DRES. Nubwo abarwayi benshi bafite umwijima umwe, abarwayi 1 kuri 5 bahari bafite impyiko zonyine. Antibiyotike niyo miti ikunze kugaragara cyane yangiza impyiko ku barwayi ba DRESS, hamwe na vancomycine itera 13 ku ijana byangiza impyiko, ikurikirwa na allopurinol na anticonvulsants. Imvune ikabije yimpyiko yaranzwe no kwiyongera kwa serumu creatinine cyangwa kugabanuka kwumuvuduko wa glomerular, kandi rimwe na rimwe byajyanye na proteinuria, oliguria, hematuria cyangwa byose uko ari bitatu. Byongeye kandi, hashobora kubaho gusa hematuria cyangwa proteinuria yonyine, cyangwa nta nkari. 30% by'abarwayi banduye (21/71) bahawe imiti yo gusimbuza impyiko, kandi mu gihe abarwayi benshi bagaruye imikorere y'impyiko, ntibyari byumvikana niba hari urukurikirane rw'igihe kirekire. Uruhare rw'ibihaha, rwaranzwe no guhumeka nabi, inkorora yumye, cyangwa byombi, byagaragaye ku barwayi ba DRESS 32%. Indwara idasanzwe yibihaha mugupima amashusho harimo kwinjirana hagati, syndrome de acute respiratory distress na pleural effusion. Ingorane zirimo umusonga ukaze, umusemburo wa lymphocytique, na pleurisy. Kubera ko UMWAMBARO W'IMBARA akenshi usuzumwa nabi nkumusonga, kwisuzumisha bisaba kuba maso cyane. Hafi ya zose zifite uruhare mubihaha ziherekejwe nizindi ngingo zikomeye zidakora neza. Muyindi suzuma rifatika, abarwayi ba DRESS bagera kuri 21% barwaye myocarditis. Myocarditis irashobora gutinda amezi nyuma yibindi bimenyetso byimyambarire igabanutse, cyangwa bikomeza. Ubwoko buratandukanye kuva myocarditis ikaze ya eosinophilique (kuvura hamwe no kuvura immunosuppressive mugihe gito) kugeza kuri myocardite ikaze ya eosinofilique (impfu zirenga 50% naho kubaho hagati yiminsi 3 kugeza 4). Abarwayi barwaye myocarditis bakunze kugaragara bafite dyspnea, kubabara mu gatuza, tachycardia, na hypotension, biherekejwe na enzyme ya myocardial enzyme, impinduka za electrocardiogram, hamwe na echocardiografi idasanzwe (nka pericardial effusion, imikorere mibi ya systolique, hypertrophy septal ventricular septal, na kunanirwa kwa biventricular). Imashusho yumutima yumutima irashobora kwerekana ibisebe bya endometrale, ariko kwisuzumisha neza mubisanzwe bisaba biopsy ya endometrale. Uruhare rw'ibihaha na myocardial ntirukunze kugaragara mu myambarire, kandi minocycline ni imwe mu miti ikurura abantu.

Sisitemu yo gutanga amanota ya RegiSCAR yu Burayi yemejwe kandi ikoreshwa cyane mugupima imyenda (Imbonerahamwe 2). Sisitemu yo gutanga amanota ishingiye kubiranga birindwi: ubushyuhe bwibanze bwumubiri hejuru ya 38.5 ° C; Indimu nini yagutse byibuze ahantu habiri; Eosinofilia; Lymphocytose idasanzwe; Rash (itwikiriye ibice birenga 50% byubuso bwumubiri, ibimenyetso biranga morfologiya, cyangwa ubushakashatsi bwamateka bujyanye na hyperensitivite yibiyobyabwenge); Uruhare rwingingo zidasanzwe; Kandi kumara igihe kinini (iminsi irenga 15).
Amanota ari hagati ya −4 kugeza kuri 9, kandi kumenya neza ko kwisuzumisha bishobora kugabanywamo ibice bine: amanota ari munsi ya 2 yerekana ko nta ndwara, 2 kugeza kuri 3 yerekana indwara zishobora kubaho, 4 kugeza 5 zerekana indwara zishobora kuba nyinshi, naho abarenga 5 bagaragaza ko basuzumye IMBARAGA. Amanota ya RegiSCAR ni ingirakamaro cyane cyane mu kwemeza ibimenyetso bishoboka kuko abarwayi bashobora kuba batujuje ibyangombwa byose bisuzumwa hakiri kare indwara cyangwa ntibabonye isuzuma ryuzuye rijyanye n amanota.

IMYENDA igomba gutandukanywa nizindi ngaruka zikomeye zuruhu, harimo SJS nindwara zifitanye isano nayo, ubumara bwa epidermal necrolysis (TEN), hamwe na impetigo ikaze ya rusange (AGEP) (Ishusho 1B). Igihe cyo gukuramo DRESS mubusanzwe ni kirekire kurenza izindi ngaruka zikomeye zuruhu. SJS na TEN bikura vuba kandi mubisanzwe bikemura bonyine mugihe cyibyumweru 3 kugeza kuri 4, mugihe ibimenyetso byimyambarire bikunda kuba byinshi. Nubwo uruhare rwa mucosal mu barwayi bambara imyenda rushobora gukenera gutandukana na SJS cyangwa ICUMI, ibikomere byo mu kanwa byo mu kanwa muri DRESS mubisanzwe byoroheje kandi ntibiva amaraso. Indwara yerekana uruhu iranga UMWAMBARO irashobora gutera catatonic ya kabiri ibisebe no gutwarwa nisuri, mugihe SJS na TEN birangwa na epidermal epidermal yuzuye hamwe nimpagarara zuruhande, akenshi byerekana ikimenyetso cya Nikolsky. Ibinyuranye, ubusanzwe AGEP igaragara amasaha kumunsi nyuma yo guhura nibiyobyabwenge kandi bigakemuka vuba mugihe cyibyumweru 1 kugeza 2. Igicucu cya AGEP kiragoramye kandi kigizwe na pustules rusange itagarukira gusa kumisatsi, itandukanye cyane nibiranga IMYENDA.
Ubushakashatsi buteganijwe bwerekanye ko 6.8% by’abarwayi bambaye imyenda bafite imiterere ya SJS, ICUMI cyangwa AGEP, muri bo 2,5% bafatwaga nk’uruhu rukabije rw’uruhu. Gukoresha ibipimo byo kwemeza RegiSCAR bifasha kumenya neza ibi bintu.
Byongeye kandi, ibisebe bisanzwe bisa nibiyobyabwenge bikunze kugaragara mugihe cyibyumweru 1 kugeza 2 nyuma yo guhura nibiyobyabwenge (kongera guhura byihuse), ariko bitandukanye na DRESS, iyi ndwara ntabwo iherekezwa na transaminase ikabije, eosinofilia yiyongera, cyangwa igihe kinini cyo gukira uhereye kubimenyetso. IMYAMBARO igomba kandi gutandukanywa n’utundi turere tw’indwara, harimo na lymphohistiocytose ya hemophagocytic, lymphoma immunoblastique T-selile lymphoma, hamwe n’indwara ikaze y’indwara.

Impuguke zumvikanyweho cyangwa umurongo ngenderwaho wo kuvura IMYAMBARO ntabwo zashyizweho; Ibyifuzo byo kuvura biriho bishingiye kumibare yo kwitegereza n'ibitekerezo by'impuguke. Ubushakashatsi bugereranya bwo kuyobora ubuvuzi nabwo burabuze, kuburyo bwo kuvura ntabwo ari bumwe.
Kuvura neza imiti itera imiti
Intambwe yambere kandi ikomeye muri DRESS ni ukumenya no guhagarika imiti ishobora gutera. Gutegura imiti irambuye yimiti kubarwayi irashobora gufasha muriki gikorwa. Hamwe nimbonerahamwe y’ibiyobyabwenge, abaganga barashobora kwandika buri gihe imiti yose ishobora gutera indwara kandi bagasesengura isano iri hagati y’ibiyobyabwenge n’ibisebe, eosinofilia, n’uruhare rw’ingingo. Bakoresheje aya makuru, abaganga barashobora gusuzuma ibiyobyabwenge bishoboka cyane ko bitera UMWAMBARA bakareka gukoresha uwo muti mugihe. Byongeye kandi, abaganga barashobora kandi gukoresha algorithms zikoreshwa muguhitamo ibiyobyabwenge biterwa nizindi ngaruka zikomeye zuruhu.

Imiti - glucocorticoide
Glucocorticoide itunganijwe nuburyo bwibanze bwo gutera umwambaro wimyenda no kuvura ibisubiramo. Nubwo ibipimo bisanzwe byo gutangira ari 0.5 kugeza kuri 1 mg / d / kg kumunsi (bipimwa na prednisone bihwanye), harabura ibizamini byo kwa muganga byerekana imikorere ya corticosteroide yo kwambara, ndetse nubushakashatsi kuri dosiye zitandukanye hamwe nuburyo bwo kuvura. Igipimo cya glucocorticoide ntigomba kugabanywa uko bishakiye kugeza igihe hagaragaye iterambere ry’amavuriro, nko kugabanya ibisebe, imboro ya eosinofil, no kugarura imikorere yingingo. Kugira ngo ugabanye ibyago byo kongera kubaho, birasabwa kugabanya buhoro buhoro urugero rwa glucocorticoide mu byumweru 6 kugeza 12. Niba igipimo gisanzwe kidakora, noneho kuvura "glucocorticoid", mg 250 kumunsi (cyangwa bihwanye) muminsi 3, birashobora gutekerezwa, hanyuma bikagabanuka buhoro buhoro.
Ku barwayi bafite IMYENDA yoroheje, corticosteroide ikora neza irashobora kuba uburyo bwiza bwo kuvura. Kurugero, Uhara n'abandi. yatangaje ko abarwayi 10 bambaye imyenda bakize neza nta glucocorticoide itunganijwe. Ariko, kubera ko bitumvikana neza abarwayi bashobora kwirinda neza uburyo bwo kuvura sisitemu, gukoresha cyane imiti ivura ntabwo byemewe nkubundi buryo.

Irinde kuvura glucocorticoid hamwe nubuvuzi bugamije
Ku barwayi bambaye imyenda, cyane cyane abafite ibyago byinshi byo guhura nibibazo (nk'indwara) biturutse ku gukoresha urugero rwinshi rwa corticosteroide, imiti yo kwirinda corticosteroid irashobora gutekerezwa. Nubwo hari amakuru yagiye avuga ko immunoglobuline yo mu maraso (IVIG) ishobora kuba ingirakamaro mu bihe bimwe na bimwe, ubushakashatsi bweruye bwerekanye ko ubwo buvuzi bufite ibyago byinshi by’ingaruka mbi, cyane cyane tromboembolism, bigatuma abarwayi benshi amaherezo bajya kwivuza glucocorticoid. Ingaruka zishobora kuba za IVIG zishobora kuba zifitanye isano n'ingaruka zayo zo gukuraho antibody, zifasha guhagarika kwandura virusi cyangwa kongera gukora virusi. Ariko, kubera dosiye nini ya IVIG, ntishobora kuba ikwiye kubarwayi bafite ikibazo cyumutima utera, kunanirwa nimpyiko, cyangwa umwijima.
Ubundi buryo bwo kuvura burimo mycophenolate, cyclosporin na cyclophosphamide. Muguhagarika ibikorwa bya T selile, cyclosporine ihagarika gene kwanduza cytokine nka interleukin-5, bityo bikagabanya kwinjiza eosinofilique no gukora ibiyobyabwenge bya T selile. Ubushakashatsi bwakorewe ku barwayi batanu bavuwe na cyclosporine n’abarwayi 21 bavuwe na glucocorticoide ya sisitemu bwerekanye ko gukoresha cyclosporine bifitanye isano n’igipimo gito cy’iterambere ry’indwara, kunoza ingamba z’amavuriro na laboratoire, ndetse no kumara igihe gito mu bitaro. Nyamara, cyclosporine ntabwo ifatwa nkumurongo wambere wo kuvura IMYENDA. Azathioprine na mycophenolate bikoreshwa cyane mubuvuzi bwo kubungabunga aho kuvura induction.
Antibodiyite za Monoclonal zakoreshejwe mu kuvura IMYENDA. Harimo Mepolizumab, Ralizumab, na benazumab ihagarika interleukin-5 hamwe na reseptor axis, Janus kinase inhibitor (nka tofacitinib), hamwe na antibodiyite zirwanya CD20 (nka rituximab). Muri ubwo buvuzi, imiti irwanya interleukin-5 ifatwa nk'ubuvuzi bworoshye, bworoshye kandi bwizewe. Uburyo bwo gukora bushobora kuba bufitanye isano no kuzamuka hakiri kare urwego rwa interleukin-5 muri DRESS, ubusanzwe iterwa na selile yihariye ya T. Interleukin-5 niyo igenga eosinofile kandi ishinzwe gukura kwabo, gutandukana, kwinjiza abakozi, gukora no kubaho. Imiti irwanya interleukin-5 ikoreshwa mu kuvura abarwayi bagifite eosinofilia cyangwa imikorere mibi y’umubiri nyuma yo gukoresha glucocorticoide.

Igihe cyo kuvurwa
Ubuvuzi bwa DRESS bugomba kuba bwihariye kandi bugahinduka ukurikije uko indwara igenda itera. Abarwayi bafite IMYAMBARO basaba ibitaro, kandi hafi kimwe cya kane cyibi bibazo bisaba ubuvuzi bukomeye. Mugihe cyo gushyirwa mubitaro, ibimenyetso byumurwayi bisuzumwa buri munsi, hakorwa isuzuma ryuzuye ryumubiri, kandi ibimenyetso bya laboratoire bikurikiranwa buri gihe kugirango harebwe uruhare rwingingo nimpinduka muri eosinofile.
Nyuma yo gusezererwa, isuzuma ryo gukurikirana buri cyumweru riracyasabwa gukurikirana impinduka zimiterere no guhindura gahunda yo kuvura mugihe. Gusubiramo birashobora kubaho ubwabyo mugihe cya glucocorticoid ikabije cyangwa nyuma yo gukira, kandi birashobora kwerekana nkikimenyetso kimwe cyangwa ibisebe byaho, bityo abarwayi bakeneye gukurikiranwa igihe kirekire kandi byuzuye.