37_lupus eritematoso sistemico pediatrico optimized

7/25/2019 37_Lupus Eritematoso Sistemico Pediatrico Optimized

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Ld dupus jrltjhctksk slstjhlek (DJU) î ugc hcdcttlc lgchhctkrlc erkglec

ca jzlkdkolc sekgkselutc j c pctkojgjsl cutklhhugltcrlc, ecrcttjrlzzctcacdd“lgtjrjsschjgtk al hkdtl krocgl ja cppcrctl j lg pcrtlekdcrj, pjr prjvc-djgzc j orcvltæ, dc eutj, dj crtlekdczlkgl j ld slstjhc husekdk-senjdjtrlek,dj sljrksj, ld rjgj, ld slstjhc gjrvksk, ld eukrj, l pkdhkgl j acddc ilkslgtjslal hkdtjpdlel cutkcgtlekrpl, trc l qucdl qujddl vjrsk cgtlojgl guedjcrl. LdDJU gjl ichilgl î bkgachjgtcdhjgtj dc stjssc hcdcttlc ajdd“caudtk ekg djstjssj hcglbjstczlkgl edlglenj j ekg dj stjssj cdtjrczlkgl al dcikrctkrlk, hcd“cpprkeelk alcogkstlek j tjrcpjutlek ajvj tjgjr ekgtk ajdd“lhpcttk ajddchcdcttlc j ajddc tjrcplc suddc erjseltc j suddk svlduppk slek j pslekdkolek.

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Î ugc hcdcttlc rcrc enj ekdplsej : pjr hldlkgj al ichilgl d“cggk. Ulstlhc enj gjodl Utctl Zgltl vl slcgk :-36 hldc ichilgl cbbjttl ac DJU jenj ld 3:-3?% ajl pczljgtl ekg DJU ciilc cvutk d“jskralk ajddc hcdcttlcaurcgtj d“lgbcgzlc. Gjl ichilgl, lg eul odl jbbjttl krhkgcdl skgk hlglhl ldrcppkrtk B=H î 5=3 j sjiijgj dc hcdcttlc pkssc hcglbjstcrsl ca kogl jtæ, î plü brjqujgtj akpk l : cggl j dc prjvcdjgzc cuhjgtc akpk dc prlhc ajecajal vltc. D“lgelajgzc j dc sjvjrltæ ajd DJU gjdd“lgbcgzlc vcrlcgk c sjekgac

ajddj vcrlj rczzj j actl prjdlhlgcrl lgalecgk ug“lgelajgzc cggucdj al 6.52ecsl pjr 366.666 lg skoojttl ecgcajsl j ug“lgelajgzc al 6.5?-6.< ecsl pjr366.666 lg skoojttl gdcgajsl, hjgtrj gjodl Utctl Zgltl î stctk kssjrvctc

ug“lgelajgzc cggucdj al 6.:5-6.26 pjr 366.666 skoojttl lg jtæ lgbcgtldj.

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Dc ecusc ajd DJU î sekgkselutc, hc î prkicildj ug“krlolgj hudtlbcttk-rlcdj ajddc hcdcttlc enj ekhprjgaj bcttkrl ojgjtlel, krhkgcdl, chiljgtcdlj lhhugkdkolel.

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Vjalctrle systjhle dupus jrytnjhctksus Cgojdk ]kdl, Olcgbrcgek Bjrrceelkdl



Rl î ugc jdjvctc ekgekracgzc al DJU trc ojhjddl hkgkzloktlel (31-:?%)j ld :-39% al pcrjgtl al skoojttl ekg DJU î cbbjttk acddc hcdcttlc. Qrc l pczljgtl ekg DJU sl kssjrvc ugc hcoolkrj brjqujgzc al hcr`jrs ojgjtlel

spjelel rlspjttk cddc pkpkdczlkgj ojgjrcdj enj lgeduakgk= NDC-I>0NDC-AY90 NDC-AY50 AS_30 ajelt al E90 ajelt al E3q0 ajelt al

E10 icssl dlvjddl al rjejttkrj-3(EY3) ajd ekhpdjhjgtk0 cdeugl cddjdl ajd

rjejttkrj Be0 pkdlhkrshl al vcrlj eltkenlgj j al rjejttkrl al eltkenlgj.

Odl krhkgl sjssucdl gkg ecuscgk ld DJU, hc pksskgk prjalspkrrj cddchcdcttlc. Dc ecrcttjrlstlec ajd pczljgtj ekg DJU, hcsenlk k bjhhlgc, î ugcrjdctlvc ajeljgzc al cgarkojgl ja ug cuhjgtk ajodl jstrkojgl. Ukgk stctl

kssjrvctl gkrhcdl dlvjddl al jstrkojgl, hc rlakttl dlvjddl al tjstkstjrkgj lgrcoczzl j rcoczzj ekg DJU akpk dc puijrtæ.L rcool udtrcvlkdjttl I ajtjrhlgcgk dc bktk-ksslaczlkgj j dc ajorcaczlkgj

ajd AGC c dlvjddk ajddj ejddudj ajddc eutj, cuhjgtcgakgj dc lhhugkoj-gleltæ ja lgauekgk d“cpkptksl ajl enjrctlgkeltl. Gjd prkejssk al cpkptkslld AGC î ajorcactk c guedjkskhl, enj skgk lhpkrtcgtl lgauttkrl ajodl

CGC. Aurcgtj d“cpkptksl dj Idjis enj ekgtjgokgk sgYGVs ekhj Yk/

UUC al :9@A, vjgokgk jspkstj suddc supjrelj ejddudcrj j ld hctjrlcdj

guedjcrj puù alvjgtcrj eksä, lhhugkojgk. L rcool udtrcvlkdjttl I lgau-ekgk d“jsprjsslkgj al LECH-3 ac pcrtj ajl enjrctlgkeltl ja cuhjgtcgk

dc prkauzlkgj al LD-> j QGBc c dlvjddk ajdd“jplajrhlaj.Ld rukdk al cojgtl lgbjttlvl gjddc pctkojgjsl ajd DJU gkg î enlcrk. Jdjvctltltkdl al cgtlekrpl cgtlvlrus al Jpstjlg-Icrr, Eltkhjocdkvlrus k Njrpjs

vlrus prkicildhjgtj rljttkgk d“cttlvczlkgj pkdledkgcdj ajddj ejddudj I

pluttkstk enj lgbjzlkgl vlrcdl spjelenj.

Cdeugl bcrhcel pksskgk ajtjrhlgcrj ug dupus k cdtjrczlkgl slhld-duplenjenj albbjrlsekgk acd dupus lalkpctlek pjr ld bcttk al jssjrj alpjgajgtl acddc prjsjgzc ajd bcrhcek j al sekhpcrlrj al skdltk cddc suc skspjgslkgj. Lbcrhcel enj plü ekhugjhjgtj pksskgk acrj hcglbjstczlkgl edlglenj jsljrkdkolenj ajd DJU skgk d“larcdczlgc, d“lskglczlaj, dc pjglelddlgc, djsudbkgchlal, l ijtc-cokglstl j odl cgtlekgvudslvcgtl. Gjd DJU vl î ug“cuhjgtctc jsprjsslkgj ajddj bugzlkgl ajl dlgbkeltl I, pklenì

cdeugj hcglbjstczlkgl edlglenj, ekhj d“cgjhlc jhkdltlec, dc trkhikeltkpjglcj dj cdtjrczlkgl ajddc ekcoudczlkgj skgk hjalctj ac cgtlekrpl j dc gjbrltj jdc vcseudltj skgk hjalctj ac lhhugkekhpdjssl. Ld guhjrk ajl dlgbkeltl I î

cuhjgtctk gjl pczljgtl ekg DJU cttlvk j ajtjrhlgc ug“cuhjgtctc prkauzlkgjal cgtlekrpl enj aæ krlolgj cdd“lpjrochhcodkiudlgjhlc. LoO cgtl-asAGC skgk

ecrcttjrlstlenj ajd DJU j pksskgk krlolgcrj ac ug“cttlvczlkgj pkdledkgcdj I-

ejddudcrj lgakttc ac vlrus k icttjrl k acddc prjsjgtczlkgj cd slstjhc lhhugltcrlk

al ekhpdjssl AGC-prktjlgc ekgtjgutl gjl guedjkskhl k gjddc erkhctlgc.D“lhpkrtcgzc ajddc bkrhczlkgj al lhhugkekhpdjssl gjddc pctkojgjsl ajd-dc hcdcttlc î tjstlhkglctc acd bcttk enj cdeugj hcglbjstczlkgl ajd DJU skgkslhldl c qujddj ajddc hcdcttlc ac sljrk enj î akvutc c lhhugkekhpdjssl,



acdd“cttlvczlkgj ajd ekhpdjhjgtk j acdd“lpkekhpdjhjgtjhlc aurcgtj djbcsl al cttlvltæ j acddc alhkstrczlkgj al lhhugkekhpdjssl gjd sljrk j gjltjssutl. Zgc pksslildj lgsubeljgtj edjcrcgej ajodl lhhugkekhpdjssl ac

pcrtj ajd slstjhc rjtlekdk-jgaktjdlcdj, gjl pczljgtl ekg DJU, î akvutc cl icssl dlvjddl ajl rjejttkrl EY3 ajd ekhpdjhjgtk suddc supjrelj ejddudcrj

j cddc prjsjgzc al rjejttkrl c icssc cbgltæ pjr LoO9 (BEo). D“cttlvczlkgjajddc ecsectc ajd ekhpdjhjgtk ojgjrc dc bkrhczlkgj al E5c, E:c enj

cttlrcgk djuekeltl j hcerkbcol j lggjsecgk l bjgkhjgl ajdd“lgchhczlkgj.

L icssl dlvjddl al E1 j E5 j dc alhkstrczlkgj al ekhpkgjgtl ajd ekhpdj-hjgtk gjl ekhpdjssl lhhugl c dlvjddk odkhjrudcrj lgalecgk d“cttlvczlkgjajddc vlc edcsslec j ajddc vlc cdtjrgctlvc ajd ekhpdjhjgtk gjddj djslkglrjgcdl. Dc ajpkslzlkgj al lhhugkekhpdjssl gjodl spczl suijgaktjdlcdlgjl pdjssl ekrklajl, puù ekgtrliulrj cdd“jgejbcdkpctlc duplec lgtjrbjrjgakekg dc elrekdczlkgj ajd dlqulak ejrjirksplgcdj.L pczljgtl ekg DJU pksskgk jssjrj gjutrkpjglel, hc dc ecpceltæ bugzlkgcdjajl gjutrkdl î gkrhcdj. Ukgk stctl ajserlttl, pjrù, ecsl al ichilgl ekg hc-dcttlc erkglec orcgudkhctksc lg eul vl î ug albjttk ajddc ecpceltæ al `lddlgo icttjrlec ajl gjutrkdl, enj ncggk cgenj hcglbjstczlkgl slhld-duplenj.

L pczljgtl ekg DJU cttlvk ncggk ugc dlgbkeltkpjglc Q ja î alhkstrctkugk snlbt acddj ejddudj Qn6 cddj Qn9 ekg prkauzlkgj al LD-1,LD-:,LD-2 jLD-36 enj suppkrtcgk d“cttlvczlkgj dlgbkeltcrlc I.

D“jsprjsslkgj al Ied-9, ug ojgj enj prkhukvj dc skprcvvlvjgzc ejddudcrj, î

cuhjgtctc gjddj ejddudj Q ajd scgouj pjrlbjrlek al skoojttl ekg DJU j ekrrjdcekg d“cttlvltæ al hcdcttlc. D“jsprjsslkgj ajddc prktjlgc Bcs î cuhjgtctc pcr-tlekdcrhjgtj gjddj ejddudj Q EA>+, ajtjrhlgcgak d“cuhjgtk ajdd“cpkptksl

al qujstj ejddudj j ugc dlgbkeltkpjglc. Zgc hutczlkgj ajd ojgj Bcs î stctcajserlttc gjl ichilgl ekg pctkdkolj dlgbkprkdlbjrctlvj, hc cppcrj jssjrj rcrcgjd DJU. Zg cddugochjgtk ajddc vltc al ejddudj prklgchhctkrlj pktrjiij

pkrtcrj cdd“cttlvczlkgj al hcdcttlc hjgtrj ug“cuhjgtctc cpkptksl pktrjiij pkrtcrj cdd“jspkslzlkgj al cgtlojgl guedjcrl gjddj idjis cpkptktlenj j gjlguedjkskhl stlhkdcgak dc bkrhczlkgj al cutkcgtlekrpl.

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Dj hcglbjstczlkgl lglzlcdl plü ekhugl ajd DJU lg jtæ pjalctrlec skgkrcpprjsjgtctj ac slgtkhl slstjhlel ekhj bjiirj, hcdjssjrj, ecdk pkg-ajrcdj, rcddjgtchjgtk gjddc erjseltc. Vuù ekhpcrlrj crtrltj ajddj pleekdjcrtlekdczlkgl j eklgvkdolhjgtk rjgcdj, hjgtrj ld edcsslek rcsn hcdcrj puùjssjrj cssjgtj gjl auj-tjrzl ajl pczljgtl.

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D“jrltjhc —c bcrbcddc‘ ajd vkdtk sl hcglbjstc ac ug tjrzk cddc hjtæ ajl pczljgtl cdd“jskralk. Î ecrcttjrlstlechjgtj slhhjtrlek, eklgvkdojgak dj



jhlgjgzj hcdcrl j dj cdl ajd gcsk, dljvjhjgtj rldjvctk j ajhcrectk. Vuùjssjrj sectjgctk acdd“jspkslzlkgj cd skdj. Cdtrj hcglbjstczlkgl eutcgjj

al tlpk hceudkpcpudcrj, pcrtlekdcrhjgtj su crjj bktkjspkstj, pksskgk

jssjrj jsprjsslkgj al vcseudltj, eksä ekhj d“jrltjhc pjrlugoujcdj, dcdlvjak rjtlekdcrj k dc pkrpkrc pcdpcildj.Dj djslkgl ajd dupus eutcgjk suiceutk lglzlcgk ekhj pcpudj enj jvkdvkgklg djslkgl cgudcrl acl ikral rldjvctl j pksskgk kssjrvcrsl suddc bceelc, sudtrkgek j suodl crtl.D“jrltjhc pjrlugoujcdj rljttj dc aldctczlkgj j dc tkrtuksltæ ajl ecplddcrl

suiugoujcdl, hjgtrj dc dlvjak rjtlekdcrj, enj sl kssjrvc pcrtlekdcrhjgtjcddj jstrjhltæ lgbjrlkrl rljttj d“cttlvltæ al hcdcttlc ja î csskelctc cddc

prjsjgzc al cgtlekrpl cgtlbksbkdlplal.Dj djslkgl alseklal skgk pkek brjqujgtl gjl ichilgl j sl kssjrvcgk plü brj-qujgtjhjgtj lg qujddl al rczzc gjrc. Ncggk ugc alstrliuzlkgj cslhhjtrlec

sud euklk ecpjddutk k suodl crtl j skgk hcglbjstczlkgl pcpudksquchksj enjoucrlsekgk ekg ctrkc, electrlzzczlkgj j cdtjrczlkgj ajddc plohjgtczlkgj

j pksskgk csskelcrsl ca cdkpjelc dkecdlzzctc. Zg“cdkpjelc ecrcttjrlzzctc acug albbusk alrcachjgtk ajl ecpjddl sl csskelc cddc hcdcttlc cttlvc.Dj djslkgl ikddksj skgk lgbrjqujgtl j ld dupus prkbugaus î stctk ajserlttkrcrchjgtj.Sucdenj ichilgk hkstrc udejrczlkgl ajddc hueksc krcdj, enj skgk lgak-djgtl, supjrelcdl j sud pcdctk aurk. Zgc stkhctltj cbtksc î plü brjqujgtj,

hc cspjelec.

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Crtrcdolj ja crtrltj skgk prjsjgtl gjddc hcoolkr pcrtj ajl ichilgl ekgDJU. D“crtrltj puù jssjrj hlorcgtj, al irjvj aurctc j eklgvkdojrj hcgl, pkdsl, okhltl, spcddj, olgkeenlc j ecvlodlj. Vuù kssjrvcrsl tjgkslgkvltj ajdakrsk ajddj hcgl j ajl pkdsl. D“crtrltj nc hlglhl cspjttl al prkdlbjrczlkgjslgkvlcdj j gkg î jrkslvc. Zg“crtrltj al tlpk Mceekua, ecrcttjrlzzctc acsuidussczlkgj rjvjrslildj akvutc c tjgkslgkvltj, sl csskelc cddc prjsjgzcal cgtlekrpl cgtl Z3YGV.

Hlcdolj ja cstjglc husekdcrj skgk ecrcttjrlstlenj ajodl stctl ceutlal hcdcttlc. L olkvcgl pczljgtl ekg DJU ncggk ug rlsenlk jdjvctk alkstjkgjerksl, spjelcdhjgtj sj skgk stctl trcttctl pjr dugok pjrlkakekg stjrklal.

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L ichilgl ekg DJU pksskgk prjsjgtcrj, g acdd“lglzlk, plcstrlgkpjglc,

cgjhlc jhkdltlec j pjrslstjgtj djuekpjglc j dc prjsjgzc al qujstj cdtj-rczlkgl lg ug ichilgk akvrjiij lhhjalctchjgtj pkrtcrj cddc rlejrecajodl CGC j ajddj cdtrj hcglbjstczlkgl ajd DJU. L pczljgtl ekg DJU

pjalctrlek pksskgk cvjrj cgtlekrpl cgtlbksbkdlplal k ug tjst pksltlvk pjr



cttlvltæ dupus cgtlekcoudcgtj j prjsjgtcrj dk stjssk rlsenlk al alskralglajddc ekcoudczlkgj ajodl caudtl.

LGQJYJUUCHJGQK YJGCDJZgc gjbrltj edlglechjgtj jvlajgtj sl hcglbjstc lg cdhjgk ld ?:% al ichilgl ekg DJU j puù jssjrj plü brjqujgtj j plü orcvj gjl ichilglenj gjodl caudtl. Vuù hcglbjstcrsl ekg jhcturlc j prktjlgurlc cdd“j-schj al rkutlgj ajddj urlgj k ekg slgarkhj gjbrktlec k lgsubeljgzc

rjgcdj ceutc.Ukttk d“cspjttk lstkdkolek sl rlekgksekgk 2 edcssl al eklgvkdolhjgtkrjgcdj gjd DJU=• Edcssj L (gkrhcdj)

• Edcssj LL (hjscgolcdj)

― LL c ajpksltl hjscgolcdl ekg HJ j LB ― LL i ajpksltl hjscgolcdl, lpjrejddudcrltæ j hctrlej hjscgolcdj• Edcssj LLL (odkhjrudkgjbrltj prkdlbjrctlvc bkecdj)

― Lgtjrjssc ― :6% supjrelj ajl odkhjrudl j ajddj cgsj ecplddcrl0

lpjrejddudcrltæ, ajpksltl hjscgolcdl j skttkjgaktjdlcdl, +/-gjerksl

• Edcssj LR (odkhjrudkgjbrltj prkdlbjrctlvc albbusc)

― Kdtrj ld :6% ajl odkhjrudl, lpjrejddudcrltæ, +/-gjerksl, +/-sjhldugj,

vcrlcildj sedjrksl, ajpksltl hjscgolcdl, skttkjgaktjdlcdl (j +/-skttk-jpltjdlcdl)

― Cspjttk wlrj dkkp cd HK

• Edcssj R (odkhjrudkgjbrltj hjhircgksc)

― Albbusk j lrrjokdcrj lspjsslhjgtk ajddc hjhircgc icscdj, ajpksltl

hjscgolcdl j skttkjpltjdlcdl• Edcssj RL (sedjrksl odkhjrudcrj)

― Lcdlgksl odkhjrudcrj, ctrkc tuiudcrj, irksl lgtjrstlzlcdj, rcrl k

cssjgtl ajpksltl LE.

Zgc odkhjrudkgjbrltj prkdlbjrctlvc albbusc sl hcglbjstc lg elrec ld 96%al tuttl l ichilgl ekg DJU skttkpkstl c ilkpslc rjgcdj.

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Î sjekgak cddc gjbrltj ekhj ecusc al hkrildltæ j hkrtcdltæ, sl hcglbjstcgjd 96-16 pjr ejgtk ajl ichilgl ekg DJU j puù jssjrj prjsjgtj cdd“jskralkajddc hcdcttlc. Dj hcglbjstczlkgl gjurkpslenlctrlenj skgk dj plü ekhuglj lgeduakgk ajprjsslkgj, albekdtæ al ekgejgtrczlkgj j al hjhkrlc j

psleksl ekg cdduelgczlkgl j pcrcgklc.

Alsturil al tlpk ekogltlvk pksskgk jssjrj prjsjgtl lg kdtrj ld :6 pjr ejgtkajl ichilgl j cakdjsejgtl ekg DJU, tjstlhkglctj acdd“cdtjrczlkgj al tjsts pslekhjtrlel0 lgkdtrj ugc hcdcttlc al dugoc aurctc puù ceekhpcogcrsl c

ug plü icssk qukzljgtj al lgtjddlojgzc.



Hcglbjstczlkgl jpldjttlenj pksskgk jssjrj keecslkgcdhjgtj d“lglzlcdjhcglbjstczlkgj al ug dupus pjalctrlek. Ukgk ojgjrcdlzzctj j plü rcrc-hjgtj bkecdl. Djslkgl lsenjhlenj ejrjircdl pksskgk jssjrj csskelctj ca

lpjrtjgslkgj, cddc prjsjgzc al cgtlekrpl cgtlbksbkdlplal k ca jhkrrcolcejrjircdj sjekgacrlc c trkhikeltkpjglc. D“JJO puù rlvjdcrj cdtjrczlkglal tlpk bkecdj k albbusk ajdd“cttlvltæ jdjttrlec ejrjircdj, dc QE, dc YHG

j d“cgolkYHG skgk utldl pjr akeuhjgtcrj j ecrcttjrlzzcrj djslkgl

bkecdl, d“jschj ajd dlqulak ejbcdkrcenlalcgk pjr jseduajrj lgbjzlkgl kjhkrrcolj suicrcegklajj, l tjsts gjurkekogltlvl pjr jvlajgzlcrj ajelt

ekogltlvl lglzlcdl.

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Dc prjvcdjgzc ajd eklgvkdolhjgtk ecralcek gjl ichilgl ekg DJU gkg î ijg ekgkselutc lg qucgtk albeldj ac alcogkstlecrj pjrenì dj cdtjrczlkgl

ecralcenj gjl ichilgl skgk spjssk sldjgtl.Dc pjrlecraltj î dc plü ekhugj ajddj hcglbjstczlkgl ecralcenj lg elrec ld56 pjr ejgtk ajl ichilgl, hc rcrchjgtj pkrtc c tchpkgchjgtk ecralcek.L ichilgl ekg DJU skgk cgenj c rlsenlk pjr d“jgakecraltj al Dlihcg-Uce`s, ecrcttjrlzzctc ac gkaudl al 3-1 hh ekg gjerksl irlgklaj ajl tjssutl

ekggjttlvl al skstjogk vcdvkdcrj. Dc vcdvkdc hltrcdj î qujddc plü ekhu-gjhjgtj lgtjrjssctc j puù skvrcppkrsl cgenj ug“jgakecraltj lgbjttlvc.Dc brjqujgzc ajddc hlkecraltj sjhirc jssjrj slhldj c qujddc ajdd“caudtkgjd 36-3: pjr ejgtk ajl pczljgtl, dc qucdj sl hcglbjstc ekg lgsubeljgzc

ecralcec ekgojstlzlc, ecralkhjocdlc j crlthlj.Actl rjejgtl lgalecgk enj l ichilgl ekg DJU ncggk ug rlsenlk cuhjg-

tctk al ctjrksedjrksl prjekej, hc dc hcdcttlc ekrkgcrlec puù gkg jssjrjjvlajgtj genì gkg rcoolugocgk d“jtæ caudtc.

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Dj hcglbjstczlkgl pdjurkpkdhkgcrl plü brjqujgtjhjgtj kssjrvctj lg jtæ pjalctrlec skgk dc pdjurltj, dj pkdhkgltl j dj jhkrrcolj pkdhkgcrl. Dksnrlg`lgo dugo, rcpprjsjgtctk acddc pjraltc al vkduhj pkdhkgcrj, ecrct-

tjrlzzctc ac ugc rlauzlkgj prkorjsslvc ajddc ecpceltæ vltcdj lg cssjgzc alirksl k vjrschjgtk pdjurlek, ekhj rlsudtctk al ug hcdbugzlkgchjgtk

ajd alcbrchhc, î stctk kssjrvctk gjl ichilgl j puù pkrtcrj c orcvj lg-subeljgzc rjsplrctkrlc.

Dc pdjurltj sl hcglbjstc lg elrec ld 56-5: pjr ejgtk ajl ichilgl ekg DJU.Vuù jssjrj prjsjgtj cd hkhjgtk ajddc alcogksl k ekhpcrlrj gjd ajekrskajddc hcdcttlc.

Dc pkdhkgltj duplec ceutc sl hcglbjstc ekg lgdtrctl j ctjdjttcslc j ekhpc-rj gjd 36-3: pjr ejgtk ajl ichilgl, hjgtrj dc irksl lgtjrstlzlcdj erkglec,

gkg sl kssjrvc gjl pczljgtl pjalctrlel. D“jhkrrcolc ceutc pkdhkgcrj î ugcajddj bkrhj plü orcvl al lhpjogk pkdhkgcrj gjd DJU ja î al rcrk rlsekgtrk



gjd ichilgk. Ul hcglbjstc ekg bjiirj, tkssj, pcddkrj, tcenlpgjc, cstjglc,jplstcssl k jhkttlsl. Cgenj d“lpjrtjgslkgj pkdhkgcrj î ugc rcrc, hc orcvj

ekhpdleczlkgj ajd DJU pjalctrlek ja î prkicildhjgtj dc ekgsjoujgzc al

rlpjtutj jhikdlj pkdhkgcrl. Jhikdlj pkdhkgcrl, lpjrtjgslkgj pkdhkgcrjj trkhiksl ajddj crtjrlj pkdhkgcrl pksskgk jssjrj djoctj cddc prjsjgzcal cgtlekrpl cgtlbksbkdlplal.

LGQJYJUUCHJGQK KEZDCYJ

Odl jssuactl rjtlglel ektkgksl skgk jsprjsslkgj al vcseudltj rjtlglec jskgk cdtchjgtj suoojstlvl al ugc alcogksl al DJU. Cdtrj hcglbjstczlkgl

keudcrl ekhprjgakgk jhkrrcolc rjtlglec, keeduslkgj ajddc vjgc ejgtrcdjajddc rjtlgc, jplsedjrltj. Dc enjrctkekgolugtlvltj sjeec, jsprjsslkgj ajddcslgarkhj al Umkorjg î rcrchjgtj rlpkrtctc gjl ichilgl ekg DJU.

LGQJYJUUCHJGQK OCUQYKLGQJUQLGCDJ

Zg“csskelczlkgj ekg pcgerjctltj î stctc kssjrvctc, hc rcrchjgtj bctcdj.Zgc hkajstc jpctkhjocdlc sl kssjrvc gjl auj tjrzl ajl ichilgl, enj puù cgenj jssjrj jsprjsslkgj al stjctksl akvutc lg pcrtj cddc tjrcplcstjrklajc.

JUCHL AL DCIKYCQKYLK

Dc hcoolkr pcrtj ajodl lgalel ajddc bcsj ceutc ajddc koksl î cuhjgtctc

gjl ichilgl ekg DJU. Sujstl ekhprjgakgk dc RJU, d“lpjrochhcodkiu-

dlgjhlc pkdledkgcdj j d“cuhjgtk ajddj c9 odkiudlgj. Dc VEY î spjsskgkrhcdj gjd DJU, hc cuhjgtc gjl ecsl al lgbjzlkgj j gjl pczljgtl ekgsljrksltl ja crtrltj.Zg“cgjhlc dljvj k hkajrctc sl hcglbjstc gjddc hjtæ ajl ichilgl ekgDJU j puù cvjrj dj ecrcttjrlstlenj ajddc cgjhlc ac hcdcttlc erkglec krljttjrj d“jhkdlsl ecusctc ac cgtlekrpl cgtljrltrkeltl. Keecslkgcdhjgtj

puù kssjrvcrsl ugc sjvjrc hcdcttlc jhkdltlec.

Zgc djuekpjglc j pcrtlekdcrhjgtj ugc dlgbkeltkpjglc (43:66/hh5) î dcecrcttjrlstlec ajd DJU ja î ekhugj gjl pczljgtl ekg hcdcttlc cttlvc. Cdeugl

ichilgl pksskgk prjsjgtcrj plcstrlgkpjglc ekg pkrpkrc j spdjgkhjocdlc,hjgtrj ugc sjvjrc gjutrkpjglc sl kssjrvc rcrchjgtj.Odl cgtlekrpl cgtlguedjk skgk prjsjgtl gjd sljrk al qucsl tuttl l ichilglekg DJU. Ld tltkdk ajodl CGC alhkstrctk ekg dc hlerksekplc ekg lh-hugkukrjsejgzc vcrlc ac 3=>6 (hcdcttlc gkg cttlvc) ca 3=:396 gjddc

hcdcttlc hkdtk cttlvc. Ld pcttjrg ajodl CGC cdd“lhhugkukrjsejgzc îal skdltk khkojgjk k pjrlbjrlek suoojrjgak dc prjsjgzc al cgtlekrplcgtl-AGC.



Ecrcttjrlstlenj ajodl cutkcgtlekrpl gjd DJU

Cgtlekrpl cgtlguedjk Cdtrl cgtlekrpl

Cgtlekrpl cgtl-asAGC Cgtlekrpl cgtl-jrltrkeltl Cgtlekrpl cgtl-YGV Cgtlekrpl cgtldlgbkeltktksslel

Cgtlekrpl cgtl-Yk/UUC Cgtlekrpl cgtl-tjssutk spjelel

Cgtlekrpl cgtl-Dc/UUI Cgtlekrpl cgtlbksbkdlplal

Cgtlekrpl cgtl-Uh Bcttkrj rjuhctklaj

Cgtlekrpl cgtl-lstkgj

Dc hcoolkr pcrtj ajl pczljgtl enj ncggk ugc pksltlvltæ pjr dupus cgtl-

ekcoudcgt ncggk cgtlekrpl cgtlecralkdlplgc. Dc brjqujgzc al cgtlekrplcgtlecralkdlplgc gjd DJU lg jtæ pjalctrlec vcrlc ac :6 cd 366 pjr ejgtkja î stctc kssjrvctc d“csskelczlkgj ekg cdtjrczlkgl ajd UGE.

Dc ajtjrhlgczlkgj ajddj ekhpkgjgtl ajd ekhpdjhjgtk î ugk ajl plülhpkrtcgtl jschl al dcikrctkrlk al cttlvltæ al hcdcttlc duplec. Vksskgkjssjrj hlsurctj spjelenj ekhpkgjgtl ajd ekhpdjhjgtk ekhj E5 j E1,

hjgtrj EN:6 rljttj d“lgtjorltæ ajddc ecsectc tktcdj ajd ekhpdjhjgtk ja î

cigkrhjhjgtj icssk lg elrec ld <6 pjrejgtk ajl ichilgl ekg gjbrltj cttlvc.

ALCOGKUL

Dc alcogksl al DJU lg jtæ pjalctrlec sl icsc suodl stjssl erltjrl ajdd“Chj-rlecg Ekddjoj kb Ynjuhctkdkoy (CEY) usctl pjr odl caudtl.

EYLQJYL AJBLGL]LKGJ

3) Ycsn hcdcrj Jrltjhc ssk, plcttk k rldjvctk codl zlokhl, enj rlspcr -hlc dj pljonj gcskdcilcdl

9) Ycsn alseklaj Vdceenj jrltjhctksj rlcdzctj ekg cajrjgtj ajsquchc-zlkgj enjrctkslec j caajgschjgtk bkddlekdcrj

5) Bktksjgslildltæ Ycsn eutcgjk cdd“jspkslzlkgj skdcrj

1) Zdejrj krcdl Zdejrczlkgl krcdl k gcskbcrlgojj, lgakdjgtl:) Crtrltj Crtrltj gkg jrkslvc enj eklgvkdoj 9 k plü crtlekdczlkgl

pjrlbjrlenj2) Uljrksltj Vdjurltj k Vjrlecraltj?) Ujogl rjgcdl Vrktjlgurlc pjrslstjgtj 7 6.: o/alj

kppurj Eldlgarl ejddudcrl

>) Ulgtkhl j sjogl gjurkdkolel Ekgvudslkgl k Vsleksl

<) Ujogl jhctkdkolel Cgjhlc jhkdltlec kppurj

Djuekpjglc 41666 hh5 lg 9 k plü keecslkgl kppurj Dlgbkeltkpjglc 43:66 hh5 lg 9 k plü keecslkgl kppurj Vlcstrlgkpjglc 4366666 hh5

36) Alskralgl lhhugkdkolel Cgtlekrpl cgtl-AGC= cgtlekrpl cgtl-AGC gctlvk c tltkdkjdjvctk kppurj

Cgtlekrpl cgtl-Uh= prjsjgzc al cgtlekrpl cgtl cgtlojgj

guedjcrj Uh kppurj Vksltlvltæ al cgtlekrpl cgtlbksbkdlplal



33) Cgtlekrpl cgtlguedjk Qltkdk jdjvctk al cgtlekrpl cgtlguedjk cddc lhhugkuk-rjsejgzc k c tjsts jqulvcdjgtl, lg cssjgzc al bcrhcelenj lgauecgk ugc slgarkhj tlpk dupus

Vjr dc alcogksl al DJU skgk gjejsscrl qucttrk k plü erltjrl, lg hcgljrcslhudtcgjc k sueejsslvc aurcgtj ld pjrlkak al kssjrvczlkgj.Zg rltcrak gjddc alcogksl puù jssjrj akvutk cdd“cssjgzc ajd tlplek rcsn hc-dcrj cdd“lglzlk ajddc hcdcttlc j cdd“jdjvctc brjqujgzc al slgtkhl cspjelel

ekhj pjraltc al pjsk, bjiirlekdc k hcdjssjrj. L ichilgl enj lglzlcdhjgtjhkstrcgk auj k trj erltjrl pjr ld DJU ajvkgk jssjrj cttjgtchjgtj sjoultl pjr dc pksslildj ekhpcrsc al cdtrj hcglbjstczlkgl j dc prkorjsslkgj vjrsk

ugc alcogksl al DJU ajgltk, enj puù rlenljajrj cdeugl cggl.Cdeugl ichilgl pksskgk prjsjgtcrj ug lglzlk ceutk al hcdcttlc ekg bjiirj, rcsn, djuekpjglc j plcstrlgkpjglc enj pksskgk bcr pjgscrj ca ugkstctk lgbjttlvk.

VYKOGKUL

Dc prkogksl ajl ichilgl j ajodl cakdjsejgtl ekg DJU, trcttctl lg hcgljrccajouctc î iukgc j dc skprcvvlvjgzc î al elrec ld 366 pjrejgtk c : cgglj ajd >5 pjrejgtk c 36 cggl. Zgc prkogksl gkg bcvkrjvkdj puù jssjrj

akvutc cddk stctk skelkjekgkhlek ajd pczljgtj, cd eklgvkdolhjgtk rjgcdjk ajd slstjhc gjrvksk ejgtrcdj.

QJYCVLC

Dc tjrcplc ajd DJU gjl ichilgl j gjodl cakdjsejgtl gkg î albbjrjgtj acqujddc ajodl caudtl, tuttcvlc gjl pczljgtl lg jtæ pjalctrlec vl puù jssjrjd“lhpcttk gjoctlvk suddc erjseltc slec j pslekdkolec al tjrcplj tcdvkdtc

gjejsscrlchjgtj coorjsslvj. Gjddj bkrhj al DJU dljvl c prjvcdjgtj ekhprkhlsslkgj crtlekdcrj j eutc-gjc, ugc tjrcplc ekg BCGU pjr ekgtrkddcrj dj hcglbjstczlkgl husekdksenj-djtrlenj j ekg d“larkssledkrkenlgc (2 ho/`o/alj) pksskgk jssjrj subeljgtl.

Icssj aksl al ekrtlekstjrklal (6.9: ho/`o al prjaglskgj) pksskgk jssjrj

utldl pjr rcoolugojrj plü rcplachjgtj ld ekgtrkddk ajddc hcdcttlc. Gjl ichilgl j cakdjsejgtl enj rlenljakgk cdtl akscool al stjrklal pjr ekg-trkddcrj d“cttlvltæ al hcdcttlc (sljrksltl, cgjhlc jhkdltlec, plcstrlgkpjglc), pksskgk jssjrj utldlzzctl czctlkprlgc, hlekbjgkdctk hkbjtld k hjtktrjssctk

cddk sekpk al rlspcrhlcrj ekrtlekstjrklal. Gjl ichilgl j cakdjsejgtl ekg ekhprkhlsslkgj rjgcdj k ejrjircdj enjrlenljakgk ugc tjrcplc coorjsslvc, l rlsudtctl hlodlkrl skgk stctl kttjgutlekg ikdl jgakvjgksl al eledkbksbchlaj (:66 ho/h9 ac cuhjgtcrj c



3 o/h9 sj ijg tkddjrctl) pjr 2 hjsl, sjoultl ac ikdl kogl trj hjsl pjr cdtrl56 hjsl. Dc tjrcplc ekg eledkbksbchlaj î lg orcak al rlaurrj lg hcgljrcsloglectlvc dc prktjlgurlc j al hlodlkrcrj dc edjcrcgej ajddc erjctlgl-

gc, rlauejgak cgenj dc prkorjsslkgj ajddj djslkgl odkhjrudcrl vjrskdc sedjrksl. Dc sleurjzzc ajddc tjrcplc ekg eledkbksbchlaj gjl ichilglgkg î ijg ajgltc, lg pcrtlekdcrj dc tkssleltæ okgcalec î hcoolkrj gjodl

cakdjsejgtl sjssucdhjgtj hcturl rlspjttk cl ichilgl prjpuijrl j dj rc-oczzj svlduppcgk rcrchjgtj lgbjrtldltæ, lgkdtrj d“usk al HJUGC rlauej

ld rlsenlk al acggk vjselecdj.L ichilgl enj skgk rjslstjgtl ca cdtrl bcrhcel lhhugkskpprjsskrl pks-skgk olkvcrsl al ugc tjrcplc ekg hlekbjgkdctk. Yltuxlhci, ug cgtlekrpk

hkgkedkgcdj enlhjrlek cgtl-EA96, utldlzzctk gjddc tjrcplc ajd DJU

ajdd“caudtk, î jbecej, lgsljhj cddc eledkbksbchlaj, lg qujl ichilgl

enj gkg ncggk rlspkstk cddj tjrcplj lhhugkskpprjsslvj ekgvjgzlkgcdl, pjrhjttjgak cgenj ld rcoolugolhjgtk al hlgkrl aksl euhudctlvj aleledkbksbchlaj.

Dc tjrcplc ajd Dupus Jrltjhctksk Ulstjhlek gjl ichilgl j gjodl cakdjsejgtl

Gkrhj ojgjrcdl=lgbkrhczlkgjcpprkeelk hudtlalselpdlgcrjrlpksk cajouctkgutrlzlkgj cajouctcusk al erjhj cgtlskdcrl

lhhuglzzczlkgj spjelcdhjgtj ekg vceelgk cgtlpgjuhkekeeleklhhjalctk trcttchjgtk ajddj lgbjzlkgl

Bcrhcel cgtlgchhctkrl gkg stjrklajl

― pjr ld trcttchjgtk ajddj hcglbjstczlkgl husekdksenjdjtrlenj

Cgtlekcoudcgtl

― sj prjsjgtl cgtlecralkdlplgc c tltkdk sloglectlvk

― celak cejtldscdleldlek c icssk akscoolk sj gkg sl skgk hcglbjstctj trkhiksl ― jpcrlgc, sjoultc ac wcrbcrlg sj sl skgk hcglbjstctj trkhiksl

Larkssledkrkenlgc ― pjr dj hcglbjstczlkgl eutcgjj j lg coolugtc codl stjrklal pjr dj hcglbjstczlkgl slstjhlenj

Oduekekrtleklal ― prjaglskgj krcdj 3-9 ho/ò/alj

― hjtldprjaglskdkgj j.v. c ikdk lglzlcdhjgtj j pkl hjgsldhjgtj pjr dc tjrcplc al hcgtj-glhjgtk gjddj bkrhj sjvjrj

LhhugkskpprjsskrlCzctlkprlgc 3-9 ho/ò/alj

Eledkbksbchlaj 3-9 ho/ò/alj pjr vlc krcdj

:66-3666 ho/h9/hjsj j.v.



Ilidlkorcbc jssjgzlcdj

- Icrscdku M, Djvy AH, Uldvjrhcg JA. Cg upactj kg enldankka-kgsjt systjhle

dupus jrytnjhctksus. Eurr Kplg Ynjuhctkd. 96350 9:=232-99.- Iruggjr NL, Nuoolgs M, @djlg-Oltjdhcg HU. Vjalctrle UDJ--tkwcras c ekh- prjnjgslvj hcgcojhjgt pdcg. Gct Yjv Ynjuhctkd. 96330 ?=99:-55.

- Ijrtslcs O@ jt cd. Jurkpjcg Djcouj Coclgst Ynjuhctlsh cga Jurkpjcg Yjgcd

Csskelctlkg-Jurkpjcg Alcdysls cga Qrcgspdcgt Csskelctlkg. Mklgt Jurkpjcg

Djcouj Coclgst Ynjuhctlsh cga Jurkpjcg Yjgcd Csskelctlkg-Jurkpjcg

Alcdysls cga Qrcgspdcgt Csskelctlkg (JZDCY/JYC-JAQC) rjekhhjgac-tlkgs bkr tnj hcgcojhjgt kb caudt cga pcjalctrle dupus gjpnrltls. Cgg Ynjuh

Als. 96390 ?3=3??3->9.

37_lupus eritematoso sistemico pediatrico optimized

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