Evidence based treatment of systemic vasculitides Текст научной статьи по специальности «Клиническая медицина»

of RHEUMATOLOGY 2

EAAHNIKH PEYMATQAOriA 2015

ANAIKOnHXH REViEW

Gepaneia auainjaiiKwv aYYeiiiiöwv ßaaiajevn ae evöei^eiq.

KwvaTavTvoq Gwjäq, XpiöTiva TaaXanäKn, Xpnaioq KouTaiaväq, Arurppioq BaaiXönouXoqMoväöa KXiviKnq AvoaoXoYiaq-PeujaioXoYiaq

B' naBoXoYiKn KXiviKr] Kai Ojwvujo EpYaainpio, laipiKr] 5<oXr] EKnA, InnoKpäTeio rNA, A9r]va

nEPIAHYH

Oi auainjaiiKeq aYYeiiiiöeq nepiXajßävouv eva eupu 0äaja voanjäiwv, nou ej^aviZouv u^nXn voanpöinia Kai 9vniöinia öiav 6ev aviijeiuniCoviai KaiäXXnXa. Iinv napouaa epYaaia snixeipn6nKe n auainjaiiKn avaaKönnan Tnq ßißXioYpa^iaq nou a0opä Tiq npoieivöjeveq 9epaneieq iwv aYYeiiiiöwv uno to npiaja vewv öeöojevwv nou a0opoüv Tn xpnan ßioXoYiKwv napaYÖvrwv Yia in 9epaneia auiwv. ria inv apinpiiiöa Takayasu Kai in YiYavioKuiiapiKn apinpiiiöa nepiYpä0eiai n KXaaaiKn 9epaneia je KopiiKoaiepoeiön Ka9wq Kai n xpnan i^v auv9eiiKwv avoaoiponoiniiKwv 0apjäKwv Kai o pöXoq iwv ßioXoYiKwv 9epaneiwv aiiq av9eKiiKeq n unoiponiäZouaeq nepiniwaeiq. Iiiq ANCA axeiiZojeveq aYYeiiiiöeq, n eniXoYn inq 9epaneiaq Ka9opiZeiai ano in ßapuinia inq npoaßoXnq Kai auZniouviai oi evöei^eiq 9epaneiaq je ßioXoYiKÖ napäYovia evavii iwv B Xej0oKuiiäpwv, o onoioq eXaße npöa0aia enianjn evöei^n Yia aa9eveiq je evepYÖ vöao (rituximab). Ta öeöojeva ainv IgA aYYeiiiiöa iwv evnXiKwv

eivai nepiopiajeva Kai npoepxoviai Kupiwq anö jeXeieq ae naiöiä. Dao a0opä in vöao Behcet, npoieivoviai 9epaneieq avaXÖY^q inq npoaßoXnq opYävwv Kai ava0epoviai veöiepa öeöojeva Yia iiq nepiniwaeiq av9eKiiKwv n unoiponiaZouawv jop0wv. ZxeiiKä je inv KpuoaQaipivaijiKn aYYeiiiiöa Kai inv oZwön noXuapinpiiiöa Yiveiai eiöiKn jveia ainv aväYKn auvepYaaiaq je nnaioXöYo Yia inv evap^n KaiäXXnXnq aviiiKnq aY^Ynq aiiq jop0eq nou auvoöeuouv xpövieq ioYeveiq Xoijw^eiq (HBV, HCV). TeXoq, auZniouviai oi evöei^eiq jn 0apjaKeuiiKÜv 9epaneiwv önwq inq nXaaja0aipeanq ae aneiXniiKeq Yia in Z^n nepiniwaeiq aa9evwv je ANCA oxeiiZöjevn n KpuoaQaipivaijiKn aYYeiiiiöa.

YnEü8uvoq aXXn^oYpa$iaq

AnMHTPi°Q BaaiAönouAoq

AvanAnpuxnQ Ka8nYnTiiQ na8oAoYiaQ-Peu|jaToAoYiaq

MoväSa KAiviKriQ AvoaoAoYiaQ-Peu|jaToAoYiaQ

B' na8oAoYiKii KAiviKri Kai O|_iwvu|_io EpYaaTripio

laTpiKii ^xoAn EKnA

InnoKpäTeio rNA

Baa. Zo^iaQ 114

115 27 A8r|va

TnA.: +30-213-2088516

Fax: +30-213-2088399

E-mail: [email protected]

Corresponding author:

Dimitrios Vassilopoulos, M.D.

Associate Professor of Medicine - Rheumatology

2nd Department of Medicine and Laboratory

University of Athens Medical School

Hippokration General Hospital

114 Vass. Sophias Ave.

115 27 Athens

Tel.: +30-213-2088516 Fax: +30-213-2088399 E-mail: [email protected]

Mediterr J Rheumatol 2015; 26(2): 18-43

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

Evidence based treatment of systemic vasculitides.

Konstantinos Thomas, Christina Tsalapaki, Christos Koutsianas, Dimitrios Vassilopoulos

Clinical Immunology-Rheumatology Unit, 2nd Department of Internal Medicine and Laboratory, Athens Medical School, Hippocration General Hospital, Athens

ABSTRACT

Systemic vasculitides comprise a wide spectrum of diseases with high mortality and morbidity when not treated appropriately. In this paper, a systematic review of the suggested treatment regimens was performed under the scope of recent data regarding the use of biologic agents for the treatment of systemic vasculitides. For Takayasu arteritis and giant-cell arteritis, classic corticosteroid therapy as well as the use of conventional synthetic disease-modifying antirheumatic drugs (DMARDs) and the role of biologic therapies for resistant or relapsing cases is discussed. In antineu-trophil cytoplasmic autoantibodies (ANCA) associated vasculitides, treatment depends on disease severity and here we discuss the indications of a recently approved biologic agent that depletes B lymphocytes (rituximab). Data on adult Immunoglobulin A (IgA) vasculitis are scarce and are mainly derived from studies in children. Regarding Behcet's disease, treatment regimens are suggested based on organ involvement and new data are presented for newer treatment options in resistant or relapsing cases. A special mention is made for the need of collaboration between the rheumatolo-gist and hepatologist in secondary virus-associated (HBV, HCV) forms of cryoglobulinemic vasculitis and polyarteritis nodosa. Finally, we discuss the indications of non drug treatments, such as plasma exchange, for life threatening cases of ANCA associated or cryoglobulinemic vasculitis.

Mediterr J Rheumatol 2015; 26(1): 18-43

of RHEUMATOLOGY 2

E Л Л H N I К H РЕУМАТОЛОПД 2015

АРТНР1Т1ЛА TAKAYASU

H apTnpÍTiSa Takayasu eívai pía aYYeiÍTiSa M^YáAou Meyé9ouq aweíwv пои e|j0avíZeTai ouvñ9wQ oe áTo-ja qAiKÍaQ KáTw twv 40 eTúv Kai xapaKTnpÍZeTai anó KOKKiwjaTúSn 0AeYJOvn tou ToixwjaTOQ thq aopTnQ Kai twv KÁáSwv thq. AiaKpívovTai 5 Túnoi aváAoYa je thv éKTaon npooßoÄnQ thq aopTnQ (jejovwjéva oto aopTiKó Tó^o éwq npooßoÄn óXriQ thq aopTHQ Kai twv KÁáSwv thq).1

Zthv пÄeюфпфía twv ao9evwv (80%) п vóooq SiaSpá-jei je unoTponiáZovTa eneioóSia evepYoú фÄeYJovпQ twv aYYeÍwv, oSnY^VTaQ othv aváYKn jaKpóxpovnQ 6epaneíaQ. H фuolкП ioTopía thq vóoou SnÄwvei óti o nepiopiojóQ thq фЛeYJovш6ouQ SiaSiKaoÍaQ oto aY-YeiaKó Toíxwja, npiv 5rpoupYn9eí onjavTiKn ßЛáßп oe auTó, oxeTÍZeTai je KaAúTepn npóYvwon O ano-TeAeojaTiKÓQ xeipiojóQ twv ao9evwv je apTnpÍTiSa Takayasu anaiTeí óxi jóvo eÄeYxo thq evepYoú ßÄá-ßnQ OTa aYYeía aAAá Kai twv xpóviwv eYKaTeoTnjévwv ßÄaßwv nou ennpeáZouv thv npóYvwon thq vóoou (ooßapn avenápKeia aopTiKnQ ßaÄßiSaQ, apTnpiaKn unépTaon).

Ta KopTiKooTepoeiSn anoTeAoúv to ßaoira okéáoq tou 9epaneuTiKoú oxñjaTOQ, ouxvá ójwq oe nepi-mwoeiQ ooßapnQ npooßoÄnQ п av9eKTiKñQ vóoou anaiTeÍTai п npoo9ñKn Kai áÄÄwv avoooKaTaoTaÄTi-kwv napaYóvTwv.

ria thv évap^n thq aYWYñQ npoTeÍveTai п xopnYnon npeóviZoÁóvnQ (П aváÄoYП Sóon jeBuÄnpeövi^^-vhq) anó tou OTójaToQ (nÍvaKaQ 1). H SiápKeia xopn-YnonQ thq ouYKeKpijévnQ sóohq npoTeÍveTai va eívai 1 jnvaQ Kai ewQ ótou uфe9oúv Ta oujmwjaTa Kai

nívaKaq 1

отп ouvéxeia ouoTqveTai oTaSiaKí jeíwon thq SóonQ. Ze nepinTwoeiQ av9eKTiKÍQ vóoou п ej0ávionQ ave-ni9újnTwv evepYeiwv anó тг| XPion twv oTepoeiSwv, ouoT|veTai n npoo9íKn avoooTpononoinTiKoú napá-YOVTa. Прштп eniAoYÍ anoTeAeí n мевотре^шп (pos П unoSopíWQ) je napáAAnAn xopÍYnon 0uAAikoú o^é-oq п n aíaGeionpívn.2

Ze nepimwoeiQ anoTuxíaQ п avTévSei^nQ xopÍYnonQ twv napanávw napaYóvTwv npoTeíveTai n xopÍYnon infliximab3,4 (xopqyn°n SKióq sníoq^qq svösi^qq) п tocilizumab5 (xopqyn°n SKióq sníoq^qq svösi^qq) п mycophenolate mofetil (xopqyn°n SKióq sníoq^qq svösi^qq) п кuкЛoфшофамí6nQ. Ze nepímwon unoTponíQ TnQ vóoou jeTá Tn SiaKoní twv oTepoeiSwv npoTeíveTai n enavévap^n TnQ aYW-YÍQ je auTá Kai oe jn avTanóKpion aKoAou9eÍTai to npwTóKoAAo TnQ av9eKTiKÍQ jop0ÍQ. ria tov npooSi-opiojó TnQ evepYÓTnTaQ TnQ vóoou éxouv npoTa9eí Ta KpiTÍpia tou Kerr (ßA. napápTnja 1). H napouoía twv TuniKúv aYYeioYpa0iKúv eupnjáTwv oe nepioxéQ nou nponYoujévwQ Sev eíxav npooßAn9ei anoTeAeí to nio a^iónioro oToixeío e^éAi^nQ Kai eniSeívwonQ TnQ vóoou.

npoKeijévou va nepiopioToúv oi aveni9újnTeQ evép-YeieQ twv oTepoeiSwv ouoTqveTai TaKTiKóQ éAeYxoQ twv eninéSwv TnQ YAuKóZnQ opoú Ka9wQ Kai twv eni-néSwv TnQ apTnpiaKÍQ níeonQ evw npoTeíveTai n Tau-Tóxpovn A^n avTiooTeonopwTiKÍQ aY^YÍQ. TéAoQ, oe eniAeYjéveQ nepinTúoeiQ ouoTqveTai n xeipoupYiKñ avTijeTúnion ooßapoú ßa9Joú oTevwjévwv aweíwv je aYYeionAaoTiKí п Tono9éTnon stents.

АРТНР1Т1ЛА TAKAYASU

ApaariKñ ouuia 056q Xopñvnons Aóan

APXIKH АГОГН

ПребУ1^оЛ0УП pos 40-60 rng/r||J£pa

Ме9иЛпребУ1СоЛ0УП П pos 32-48 mg/nispa

AN0EKTIKH NOIOI H

ÜYIANEZIA ITA К0РТ1К01ТЕР0Е1ЛН

1n eniÁoyq

Мсвотрс^атп pos n sc 15-25 mg/sßöoiaöa

AZa9eionpivn pos 2 mg/kg/nispa

2n eniÁoyq

Infliximab* iv 3-10 mg/kg avá 4-8 EßöoiäösQ

Tocilizumab* iv 8 mg/kg/|nva

Mycophenolate mofetil* pos 2 gm/nispa

КикЛофшофаи^бп pos 2 mg/kg/n|spa

YnOTPOniAZOYIA NOIOI

(иста тп б1акопп тп^ apxiKñQ avuvñQ)

ПребУ1^оЛ0УП pos 40-60 mg/nispa

Ме9иЛпребУ1СоЛ0УП П pos 32-48 mg/nispa

* Xopiynon eKTóQ eníonjnQ évSei^nQ (off-label)

rirANTOKYTTAPIKH (KPOTAOIKH) APTHPITIAA

H YiYavTOKUTTapiKn apTnpiTiSa eivai n ouxvoTepn Mop-0H ouornMaTiKHQ aYYeiiTiSaQ nou npooPaAAei apTn-pieQ Meoaiou Kai MeYaAou meye9ouq oe evnAiKeQ avw twv 50 eTwv. H xpovia 0AeYMOvq a0opa Tnv aopui Kai touq KupiwQ KAaSouQ TnQ Me iSiaiTepn npoTiMnon oe KAaSouQ TnQ e^w KapwTiSaQ (eninoAnQ KpoTa<t>iKn apTnpia, yAwooikh apTnpia) Kai TnQ eow KapwTiSaQ apTnpiaQ (o09aAMiKn apTnpia), oSnYwvTaQ oe noiKi-AnQ PapuTnTaQ KAiviKa ouMnTwMaTa. H anwAeia TnQ opaonQ ouMPaivei oto 15-20% twv nepinTwoewv Kai xpHZei eneiYouoaQ 9epaneuTiKnQ avTiMeTwnionQ. H npooPoAq TnQ aopTHQ Kai KAaSwv TnQ (YiYavToKUTTa-piKH apurpTiSa MeYaAwv aYYeiwv) SiaSpaMei ouvn9wQ unoKAiviKa oSnYwvTaQ otov oxnMaTioMo apTnpiaKwv aveupuoMaTwv Me anwTepn eninAoKn tov SiaxwpioMo Kai unv pn^n auTwv oe eva MiKpo nooooro twv ao9evwv. Ta KopTiKooTepoeiSn anoTeAouv tov aKpoYwviaio Ai9o TnQ 6epaneuTiKnQ aYWYHQ unQ YiYavToKUTTapiKHQ apTnpiTiSaQ eAeYxovraQ anoTeAeoMaTiKa Ta ouMnTw-MaTa TnQ ouoTnMaTiKnQ 0AeYMovnQ aAAa Kai npoAaM-PavovraQ movimeq PAaPeQ oTnv nAeio^n^ia twv ne-pimwoewv (n.x. anwAeia opaonQ). To 9epaneuTiKo oxnMa Ka9opiZeTai ano Tnv napouoia eninAeYMevnQ n Mn npooPoAnQ (nivaKaQ 2).

Ze MH eninAeyMevn YiYavxoKUTTapiKn aprnpixiSa

npoTeiveTai n evap^n aYWYHQ Me npeSviZoAovn n ava-AoYn Soon Me9uAnpe6viZoAovnQ ano tou oT6MaToQ Yia xpoviKo SiaoTnMa 1 MnvoQ ewQ otou u0e9ouv Ta ouMnuwMaTa Kai KaToniv ouvioTaTai oraSiaKn Meiwon TnQ SoonQ.

nivaKaq 2

Ze nepinTwoeiQ onou exouMe eneiYouoeQ Kai anei-AnxiKEQ KaxaoxdoeiQ, onwQ uno e^eAi^n anwAeia opaonQ H napoSiKn aMaupwon opaonQ, npoTeiveTai n xopnYnon woewv Me9uAnpeSviZoAovnQ Yia 3 nMe-peQ Kai oTn ouvexeia npeSviZoAovn (n avaAoYn Soon Me9uAnpeSviZoAovnQ) Me MeYioTn Soon 60 mg/nM. Me Tnv eniTeu^n eAeYxou twv ouMnTwMaTwv ouornveTai npooSeuTiKn Meiwon TnQ SoonQ.2,6 H kAivikh ouMnTwMaToAoYia oe ouvSuaoMo Me Tnv na-paKoAou9non twv epYaoTnpiaKwv napaMeTpwv (TKE, CRP) Ka9opiZouv tov pu9Mo MeiwonQ TnQ SoonQ uwv oTepoeiSwv, av Kai otoxoq, eni eAeYxou TnQ vooou, eivai n SiaKonn TnQ 9epaneiaQ MeTa to nepaQ 18-24 Mnvwv.

Ze nepinTwoeiQ av9eKTiKnQ vooou n ep^dvionQ na-pevepYeiwv ano Tn xpnon uwv KopTiKooTepoeiSwv, npoTeiveTai n npoo9nKn avoooTpononoinTiKou napa-YovTa onwQ oi KaTw9i:

- MeBoxpeidxn pos n SC Me TauToxpovn xopnYnon 0uAAiKou

- aZaGeionpivn

- tocilizumab4,5 (xopnYnon ektoq enionMnQ evSei-frQ)

Ze nepinTwon unoxponnQ TnQ ouMnxwMaxoAoYiaQ

npoTeiveTai n enavevap^n H n au^non TnQ SoonQ uwv xopnYouMevwv KopTiKooTepoeiSwv. Ze Mn avTanoKpi-on, aKoAou9eiTai to npwToKoAAo TnQ av9eKTiKnQ Mop-0HQ (PA. avwTepw).

npoKeiMevou va nepiopioTouv oi aveni9uMnueQ evep-YeieQ twv oTepoeiSwv ouoTnveTai o TaKTiKoQ eAeYxoQ twv enineSwv TnQ YAuKoZnQ opou Ka9wQ Kai twv eni-

nrANTOKYTTAPIKH (KPOTAOIKH) APTHPITIAA

ApaoriKn ouuia OSoq XopnvnoHG Aoan

A. MH EnInAErMENH NOIOI

npeSviZoXovn pos 40-60 rng/r||J£pa

Me9uXnpe6viZoX6vn n pos 32-48 mg/n|£pa

B. NOIOI AnEIAHTIKH rIA THN OPAIH (Yno e^eXiin anwXeia n napoSiKn aMaupwon opaonQ)

Me9uXnpe6viZoX6vn npeSviZoAovn iv KAI ITH IYNEXEIA pos 1 gr nispnoi^Q Yia 3 nispsQ 1 mg/kg/nispa

r. AN0EKTIKH NOIOI H AYIANEHIA ITA KOPTIKOITEPOEIAH

Me9oxpe£dxn AZa9eionpivn Tocilizumab* pos n sc pos iv 15-25 mg/sp5o|a5a 2 mg/kg/n|spa 8 mg/kg/inva

A. YnOTPOnIAZOYIA NOIOI (Mexd xn SiaKonn xnQ apxiKnQ aywyHQ, A)

npeSviioXovn pos 40-60 mg/n|spa

Me9uXnpe6viCoX6vn n pos 32-48 mg/nispa

* XopnYnon ektoq enionMnQ svSei^nQ (off-label)

of RHEUMATOLOGY 2

E Л Л H N I К Н РЕУМАТОЛОПА 2015

néSwv thq артпр1акпс níeonQ evw npoTeíveTai п Tau-Tóxpovn АпФп avTiooTeonopwTiKnQ aYWYnQ. EninÁé-ov, п прообпкп avTiaiMoneTaAiaKnQ aY^YnQ (aonipívn 75-100mg/nMepnoíWQ), eKTÓQ twv nepinTwoewv nou unápxei avTévSei^n, 0aíveTai óti éxei eninpóo9eTa 9eTiKá anoTeÁéopaTa oTnv avTi0AeYMovú6n Spáon twv orepoeiSwv.

ANCA АГГЕ11Т1ЛЕ! (GPA, MPA)

Oi oxeTiZópeveQ pe ANCA aYYeiíTiSeQ anoTeÁoúv vo-onpaTa pe noAuouoTnMaTiKn npooPoAn nou характп-píZovTai anó véKpwon Kai 0AeYpovn twv piKpwv Kupí-wq aYYeíwv. nepiÁapPávouv Tnv KOKKiwpáxwan Me noÁuaYYeiÍTi6a (npwnv vóooq Wegener, GPA), тп ml-кроакоткп noAuaYY£iÍTi6a (MPA) Kai тпv nwoivo-Ф1Л1КП KOKKiwMáxwan Me noAuaYYeiÍTi6a (пpWпv oúvSpopo Churg-Strauss, EGPA). A0opoúv áTopa Kai twv 5úo 0úAwv pe peYaЛúтepп eпíптwoп otiq пÁlкÍeQ 65-75 eTwv. Ta ópYava nou npooPáÁÁovTai ouxvó-Tepa eívai to avwTepo Kai KaTwTepo avanveuoTiKó, oi ve0poí, to Séppa, oi o09aApoí Kai Ta nep^epiKá veúpa. XapaктпpíZovтal anó ифп^0 nooooTó unoTpo-nwv aкópп Kai peTá тпv enn-eu^ nÁnpouQ úфeoпQ. H пpóYvwoп Ka9opí^eTai KupíwQ anó t^v пÁlкÍa tou ao9evoúQ Kai тп napouoía п PП veфplкПQ npooPoÁnQ. H eкгípпoп тпQ evepYóT!TaQ Kai PapúTiTaQ тпQ vóoou YÍveTai pe Ta epYaÁeía BVAS (ВЛ. Пapápтпpa 2).

nEPIOPIZMENH NOZO!

H 9epaneía ao9evwv pe nepiopiopév! vóoo nepiÁapPávei to ouvSuaopó KopTiKooTepoei5wv Kai evóQ avo-ooтpoпoпolптlкoú фappáкou (nívaKaQ 3). H npeSvi-ZoAóvn (П aváЛoYП 6óoп pe9uÁпpe6vlZoÁóvпQ) xop^ YeÍTai apxiKá anó tou oTópaToQ pe péYloтп 6óoп Ta 60 mg/ipépa, pe oraSiaKn peíwo^ Ztóxoq тпQ 9e-paneÍaQ 9a npénei va eívai п peíwo! тпQ 6óoпQ twv KopTiKooTepoeiSwv oTa 15 mg/пpépa otouq 3 pnveQ, Kai oTa 5-10 mg otouq 6 pnveQ.7 Anó nÁeupáQ twv avoooтpoпoпolптlкwv фappáкwv, прйтп eniÁoYñ eívai п Me8oTpe£áTn pe пapáЛЛпAп XopпYПOП фuЛЛlкoú o^éoQ. Дeúтepп eniÁoYñ eívai п XopпYПOП rou mycophenolate mofetil (eKTóQ eпÍoп-PПQ év6el^ПQ).

nívaKaq 3

ZOBAPH/rENIKEYMENH NOZOZ

H 9epaneía ao9evwv pe ooPapп/Yevlкeupévп ANCA aYYeiíTiSa nepiÁapPávei Súo фáoelQ: тпv apxiKñ 8epa-neía ефóбou/епaYИYПQ Kai тп 8epaneía ouvTnpn-

ohq nou aKoÁou9eí (nívaKaQ 4). H пpWтп фáoп nepiÁapPávei ev6oфÁéPleQ woeiQ pe-9uÁпpe6vlZoÁóvпQ, Kai aKoÁoú9wQ xopпYП0п npeS-viZoÁóv!Q anó tou oTópaToQ п ev6oфÁePíwQ. ria тп oTaSiaKñ eÁáттwoп t^q 6óoпQ twv KopTiKoorepoei-Swv, ioxúei óti Kai oтпv пeploplopévп vóoo. !тп фáoп auTñ, п X0PПYП0П rwv KopTiKooTepoei5wv oфeÍÁel va ouvSuáZeTai pe кuкЛoфшoфaмíбn ñ rituximab.8 AvaÁuTiKóTepa, п кuкÁoфwoфapí6п pnopeí va xo-pПYП9eÍ oe ev6oфÁéPleQ woeiQ п anó tou oTópaToQ. 'OTav eniÁéYeTai п ev6oфÁéPla xopпYП0п ouvioTáTai П oupпÁпpwpaтlкп X0PПYП0П ev6oфÁePíwQ MESNA wq пpoфúÁa^п évavTi тпQ aipoppaYiKñQ KuoTíTiSaQ.9 H 6óoп тпQ кuкÁoфwoфapí6пQ TpononoieíTai ava-áóywq тпQ пÁlкÍaQ, T!Q veфplкпQ ÁeiToupYíaQ Kai tou api9poú twv ÁeuKwv (PÁ. Пapápтпpa). H SiápKeia тпQ aYwYñQ eфó6ou pe кuкÁoфwoфapí6п eívai 3-6 pnveQ. EvaÁÁaKTiKñ eniÁoYñ пpWтпQ YPappñQ Yia W apxi-кп 9epaneía eфó6ou/eпaYWYПQ eívai п ev6oфÁéPla X0PПYП0П Rituximab.10,11 Дeuтepeúouoа eпlÁ0YП Yia тп 9epaneía eфó6ou/eпaYWYПQ eívai п X0PПYП0П mycophenolate mofetil anó tou oTópaToQ.12,13 H ouYKeKpipév! 9epaneía xopПYeÍтal eKTóQ eпÍoпpпQ évSeiíflQ rou oKeuáopaToQ.

KaTá тп 9epaneía eфó6ou/eпaYWYПQ Kai oe ao9eveíQ pe:

- TaxéwQ e^eÁlooópevп oпelpapaтoveфpÍтl6a (KpeaTi-vív! opoú > 5.6 mg/dl)

- кuфeÁl6lкп aipoppaYía п

- eпlкaÁuптópevп vóoo pe oúvSpopo évavTi PaoiraíQ pepPpávпQ (anti-GBM)

ouvioTáTai п évap^ пÁaopaфaÍpeoпQ.14 H 6óoп тпQ пÁaopaфaÍpeoпQ eívai 1 - 1.5 oáikoú óykou nÁáopaToQ (TPV) avá ouveSpía Kai п ouxvóтптá тпQ pía ouveSpía пpepпoÍWQ п pépa nap' пpépa (ava-áóywq év6el^ПQ). H пÁaopaфaÍpeoп npénei va Yíve-Tai oe e^eiSiKeupéva vooпÁeuтlкá KévTpa pe epneipía oтпv eфappoYП тпQ pe9ó5ou. ZupпÁпpwpaтlкá, oi ao9eveíQ ouvioTáTai va ÁapPá-

ANCA avveiiTLSec (GPA/MPA)

^paoTiKn ouoía npeSviZoAóvn

Ме8отре^атп Mycophenolate mofetil*

OSÓQ XopñYHonQ

nEPIOPIZMENH NOZOZ

pos

KAI ¿va anó та napaKáTu: 1n emXoYn

pos n sc 2n emXoYn

_pos_

^óo^

1 mg/kg/nnépa

15-25 mg/sp5opá5a 2 gm/niJspa

* X0pпYП0П eKróQ eпÍ0ПPПQ évбel^ПQ (off-label)

vouv xnjeionpo0uXa^n evavii Pneumocystis jirovecii je Tpije9onpijn/oouX0aje9oi;aZöXn anö tou aiöja-Toq Ka9öXn in 5iäpKeia TnQ aYWYHQ je Kui<Xo<t>wa0a-jiSn n rituximab Ka9wq Kai avTioaieonopwTiKn aYWYn. H SeüTepn <t>äan nepiXajßävei in 9epaneia auvinpn-anq. Qq ysvikh apxn, n 9epaneia auvrnpnanQ npenei va SiapKei TouXäxiaTov 18-24 jnveq jeiä Tnv eni-Teu^n ü0eanQ. npwin eniXoYH eivai n aia8eionpivn anö tou öTöJaтoQ- EvaXXaKTiKä, jnopoüv va xopnYn-9oüv mycophenolate mofetil anö tou aiöjaioQ,15 rituximab evSo0Xeßiwq16,17 n Me8oTpe£äTn anö tou OTöjaTOQ n unoSopiwq.8

Oi jeiZoveq unoiponeq (ßX. napäpinja 2) avTijeTW-niZoviai je Ta axnjaia nou npoieivoviai ain 9epa-neia e0ö6ou. EiSiKä ötouq aa9eveiQ je unoiponn Kai je Ta jexpi Twpa SeSojeva anö tiq TuxaionoinjeveQ jeXeieq, to rituximab unepiepei TnQ KuKÄo0wa0aji-SnQ.19 Oi eXäaaoveQ unoiponeq (ßX. napäpinja 2) avTijeTwniZovTai je Tpononoinan TnQ SöanQ twv nSn Xajßavojevwv KopTiKooTepoeiSwv Kai avoaoKaia-aTaXTiKwv.

nivaKaq 4

ANCA avveiiTiSec (GPA/MPA)

ApaoTiKn ouoia OSöc Xophvnonc Aöon

ZOBAPH/fENIKEYMENH NOIOI

©EPAnEIA EOOAOY/EnAfQrHI

Me8uXnpe6viZoXövn iv 7-15 mg/kg npspnaioic Yla 3 npspsc

npeöviZoXovn pos KAI eva anö ra napaKÖru: 1n eniXoYh 1 mg/kg/npspa

KuKXo$wa$apiSn pos 1,5-2 mg/kg/npspa |

KuKXo$wa$apiSn iv 15 mg/kg avä 2-3 sßöopäösc

Rituximab iv 375 mg/m2 avä sßöopäöa yia 4 sßöopäösc n

Rituximab iv 2n eniXoYh 1 gm Kai snaväAn^n psTä 2 sßöopäösc

Mycophenolate mofetil* pos 2 gm/npspa

Eäv ouvunäpxei eva anö Ta Kärw8i:

1. TaxswQ e^eAiooö^evq onsipayamvsQpmöa

2. KutpsAiöiKq ai^oppaYia

3. napouoia anti-GBM avnowjjaTwv Töts, smuMov: nXaoMa^aipeon

©EPAnEIA IYNTHPHIHI

1n eniXoYh

AZa8eionpivn pos 2n eniXoYh 2 mg/kg/npspa

Me8oTpe^äTn pos n sc 15-25 mg/sßöopäöa

Mycophenolate mofetil* pos 2 gm/npspa

Rituximab* iv 500-1000 mg Ka8s 6 pnvsc

©EPAnEIA YnOTPOnHI

MeiZuv unorponq

Me8uXnpe6viZoXövn iv 7-15 mg/kg npspnaioic Yia 3 npspsc

npeöviZoXovn pos KAI eva anö ra napaKÖru: 1n eniXoYh 1 mg/kg/npspa

Rituximab iv 375 mg/m2 avä sßöopäöa Yia 4 sßöopäösc; n

Rituximab iv 2n eniXoYh 1 gm Kai snaväAn^n psTä 2 sßöopäösc

KuKXo$wo$apiSn pos 1,5-2 mg/kg/npspa n

KuKXo$wa$apiSn iv 3n eniXoYh 15 mg/kg avä 2-3 sßöopäösc

Mycophenolate mofetil* pos 2 g/npspa

* XopnYnan eKiöq enianjnq svSei^nQ (off-label)

of RHEUMATOLOGY 2

E Л Л H N I К Н РЕУМАТОЛОПД 2015

НШ^ФШ^ KOKKIfiMATfilH ME ПОЛУАГГЕ11Т1ДА (Iuv6pouo Churg Strauss, EGPA)

H nwolvoфlAlкп кокк1шратшоп HE noAuaYYeiiTiSa eivai П nAeov onavia ano tiq ANCA aYYeiiTiSeQ. Проквпа Yia aYYeiiTiSa HiKpoU Kai Heoaiou eUpouQ aYYeiwv Kai xapaKTnpiZeTai ano veKpwTiKn KoKKiwHaTwSn фAeY-Hovn he п^омоф^кп SinQnon oe ao6eveiQ he aoQHa Kai nwolvoфlAía evw ouxvn eivai Kai n napouoia pivi-kwv noAunoSwv. Ta ANCA avTiowHaTa eivai QeTiKa KupiwQ oe aoQeveiQ не veфplкп npooPoAn.20 OnwQ Kai otiq unoAoineQ ANCA aYYeiiTiSeQ, n Qepa-neia aoQevwv не п^^оф^^! KoKKiwHaTwon не noAuaYYeiiTiSa KaQopiZeTai ano Tn PapU^Ta TnQ vooou (nivaKaQ 5). Me Paon auT|, oi aoQeveiQ SiaKpivovTai oe SUo KaTnYopieQ:

- Ze aoQeveiQ не £K6nAwaeiQ aneiAnxiKeq Yia тп

Zwn П тп AeiTOupyia Opyavwv-CTTOXWV (n.x. кuфeAl-

Sik| aiHoppaYia, AeuKwHaToupia >1 gm/24h п KpeaTi-vivn >1.57 mg/dl, HuoKapSionaQeia, ooPapn npooPo-A| KevTpiKou п пeplфeplкoй veupiKoU ouoTiHaToQ, aiHoppaYia/ioxaiHia YaoTpevTepiKoU п naYKpeaTmSa)

- Ze aoQeveiQ xwpiQ £K6nAwaeiQ aneiAnTiKeq yia тп Zwn П тп AeiToupyia opyavwv-CTTOxwv.

ZTnv npwTn KaTnYopia (aoQeveiQ не eKSnAwoeiQ anei-AnTiKEQ Yia Tn Zw| П Tn AeiToupYia opYavwv-oToxwv), n Qepaneia SiaKpiveTai oe SUo фaoelQ: Tnv apxiKn Qepaneia eф6Sou/eпaYWYПQ Kai Tn Qepaneia ouvt|-pnonQ nou aKoAouQei. H Qepaneia eф6Sou/eпaYWYПQ nepiAaHPavei KopTiKooTepoeiSn Kai ouYKEKpiHeva npeSviZoAovn п iooSUvaHn Soon HeQuAnpeSviZoAo-vnQ, не otoxo Tnv eAaTTwon TnQ SoonQ oTa 15 mg/ nH^pa otouq 2-3 hiveq oe ouvSuaoHo не кuкAoфw-oфaнíSn.8 ria tiq oSoUq xopnYnonQ, tiq SooeiQ Kai Tnv napaKoAoUQnon twv aoQevwv nou AaHPavouv kukAo-фwoфaнíSn ioxUei 6ti Kai Yia tiq unoAoineQ ANCA aYYeiiTiSeQ. H SeUTepn фaon nepiAaHPavei Tn Qepaneia ouvT|pnonQ. npwTn eniAoY! eivai n aZaQeionpivn evw evaAAaKTiKa HnopoUv va xopnYnQoUv mycophenolate mofetil ano tou oToHaToQ п HeQoTpe^aTn ano tou oToHaToQ п unoSopiwQ.8

Ze avQeKTiKEQ nepinTwoeiQ п oe nepinTwoeiQ onou avTeSeiKvuTai n xopnYnon кuкAoфwoфaнíSnQ, Hnopei va xopnYnQei rituximab evSoфAePíwQ.21'22 ZTn SeUTepn KaTnYopia (aoQeveiQ xwpiQ eKSnAwoeiQ aneiAnTiKEQ Yia Tn Zwn П Tn AeiToupYia opYavwv-oTo-xwv), n Qepaneia eivai Ta KopTiKooTepoeiSn не oTaSi-aK| eAaTTwon TnQ SoonQ. Ze nepinTwoeiQ aSuvaHiaQ eAaTTwonQ TnQ SoonQ, eнфavlonQ napevepYeiwv ano Tn xopnYnon KopTiKooTepoeiSwv п unoTpon|Q TnQ vooou ouvioTaTai n npooQ|Kn aZaQeionpivnQ ano tou oToHaToQ, mycophenolate mofetil ano tou oToHaToQ п HeQoTpe^aTnQ ano tou oToHaToQ п unoSopiwQ.

IgA АГГБ11Т1ЛА (npwnv порфира Henoch-Schonlein)

H IgA aYYeiiTiSa eivai n ouxvoTepn HopфП aYYeimSaQ oTnv naiSiKn nAiKia, evw nAeov ouxva avaYvwpiZeTai Kai wq ouxvo aiTio aYYeiiTiSaQ Kai otouq eviAikouq. npoKeiTai Yia aYYeiiTiSa HiKpwv aYYeiwv nou xapaKTn-piZeTai ano evanoQeon IgA avoooouHnAeYHaTwv oto ToixwHa twv aYYeiwv. Ta nAeov ouxva npooPePAnHe-va opYava eiva to SepHa, to YaoTpevTepiKo, oi veфpoí Kai oi apQpwoeiQ. Ze nooooTo ewQ Kai 30% avaYvwpiZeTai ekAutikoq napaYovTaQ, onwQ nponYnQeioa AoiHw^n П A^n фapнaкwv. H npoYvwon TnQ vooou KaQopiZeTai ano Tn PapUTnTa TnQ veфplкпQ npooPo-A|q (AeuKwHaToupia, eniPapuHevn veфplкп AeiToupYia П napouoia HnvoeiSwv oxnHaTioHwv oTn Ploфía ve-фpoй).23

H Qepaneia aoQevwv не IgA aYYeiiTiSa KaQopiZeTai ano Tn PapUTnTa TnQ vooou (nivaKaQ 7). Me Paon auT|, oi aoQeveiQ SiaKpivovTai oe SUo KaTnYopieQ:

- XwpiQ £K6nAwaeiQ апеЛпт^ед yia тп £ип П тп A£lToupYÍa opyavwv-CTroxwv: пopфйpa, apQpaAYi-eq п apQpiTiSa, veфpíтlSa не фuoloAoYlкп AeiToupYia п npwTe'ivoupia ewQ 0.5-1 gm/24h.

- Me £K6nAuo£iQ aпelAnтlк¿Q Yia тп Zwn П тп Aei-TOupYia opYavwv -стт6xwv: veфpíтlSa не ennpea-oHevn veфplкп AeiToupYia, npwTe'ivoupia > 1 gm/ 24h, TaxewQ e^eAioooHevn oпelpaнaтoveфpíтlSa (Hnvo-

nivaKaQ 5

HыolvoфlAlкП кoккlымaтыon ue пoAuavveliтlбa (EGPA)

ApaoriKn ouaia Odoq XopnvnanG Aoan

XwpiQ екбпХыае^ aпelAnтlкcQ Yia тп Zwn П тп AeiroupYia opYavwv*

ПpeбvlZoA6vп pos 40-60 mg/пиера

MeвuAпpeбvlZoA6vп п pos 32-48 mg/n^spa

AN0EKTIKH NOIOI H ЛYIANEHIA ITA KOPTIKOITEPOEIЛH

Meвoтpe^aтп AZaвeюпpivп Mycophenolate mofetil** pos n sc pos pos 15-25 mg/sp6. 2 mg/kg/npspa 2 g/npspa

nivaKaç 6

HuoivoAiAiKn KOKKiuuâxuon ue noAuayyeiiTiSa (EGPA)

Me ekShAmoeiq aneiXnTiKSç yia тп СыП h тп Acixoupyia opvavwv

AvTcvSciKvuTai n xophvnon KUKAo0Mo0aui5nc;

OXI

PhYno

NAI

0ЕРАПЕ1А EФOAOY/EПAГQГHI

ApaoTiKh ouoia Oôôç Xophvnonc Aôon ApaoTiKh ouoia Oôôç Xophvnonc Aôon

Me9uXnpe6viÇoXovn iv 7-15 mg/kg r|pspr|aitt>ç yia 3 npspsc Me9uAnpe6viZoAovn iv 7-15 mg/kg npspnoioiç yia 3 npspsç

npeôviÇoXovn pos 0,5-1 mg/kg/ npspa npeôviÇoXovn po 0,5-1 mg/kg/ npspa

KAI ¿va anô та паракаты: KAI ¿va anô Ta napaKÖrn:

KuKAo0uo0auî6n pos 1,5-2 mg/kg/ npspa Rituximab** iv 375 mg/m2 avä sßöopäöa yia 4 sßöopäösc

KuKAo0uo0auî6n iv 15 mg/kg avä 2-3 sßö. Rituximab** iv 1 gm Kai sпaväЛnФn psTä 2 sßöopäösc

©EPAnEIA IYNTHPHIHI

ApaoTiKh ouoia Oôôç Xophvnonc Aôon

AZa9eionpivn pos 1-2 mg/kg/npspa

Me9oTpeÇâTn pos n sc 15-25 mg/sßö.

Mycophenolate mofetil** pos 2-3 g/npspa

AN0EKTIKH NOIOI

Rituximab* Rituximab*

iv iv

375 mg/m2 avä sßöopäöa yia 4 sßöopäösc 1 gm Kai ЕпауоЛпФп psTä 2 sßöopäösc

* кифеЛ1б1кп aijoppaYÎa, Xeui^jaroupia > 1 gm/24h п Kpsaiivivn > 1.57 mg/dl, миокарбюпа0Е1а, aoßapn npoaßoXn kevtpikoù п пер1фер1кои veupimù аиатпматод, aijoppaYia/iaxaijia Yaarpevrepirnu п паукреаттба

** Хорпупап EKTOç eraanjnQ évôei^nQ (off-label)

eiôeiç oxnjaTiajoi ae > 50% twv aneipa|jâTwv), ve-фpwalкô aùvôpojo, aijoppaYÎa nenTiKoù, кифеЛ101кп aijoppaYÎa, eYкeфaÀlкП aijoppaYÎa. Zrnv прштп raTriYopia (aa9eveiç xwpiç erönAwaeiQ aпelЛптlкéQ Yia тп ZW п тп XeiToupYÎa opYâvwv-ато-Xwv), n öepaneia eivai rajpiwç uпoaтпрlктlкП je anAâ avaXYHTira. Ze nepinTûaeiç je evTovôTepa aujmû-jaTa nou eniÀÉYeTai n XoрПYnan кopтlкoaтepoel6wv, auviorwvTai xajnXéç éwç jéTpieç ôôaeiç npeôviZoAô-vhq (< 30 mg/njépa) п iaoôùvajn ôôan je9uAnpe6vi-ZoXôvnQ je YPПYoрn jeiwan thq ôôanQ. Zth ôeûTepn кaтnYoрía (aa9eveiç je erönAwaeiQ aпelЛnтlкéQ Yia тп ZW п тп XeiToupYÎa opYâvwv-ато-Xwv), n ßaan thq 9epaTOÎaç eivai Ta кopтlкoaтepoel-5п rai auYкeкрlJéva пpe6vlZoÀôvn (jéYiaTn ôôan: 60 mg/njépa) п iaoôùvajn ôôan Je9uÀпpe6vlZoÀôvnQ je aтa6laкП jeiwan thq ôôanQ. Ta кopтlкoaтepoel6П Jпopoûv va auvôuaaToûv кal ae auvôuaajô je avo-aoкaтaaтaЛтlкâ, av кal JlкpéQ jn тuxaюпolnJéveQ je-XéTeç ôev éxouv Te^npiwaei aaфéQ ôфeЛoQ.24 Прштп eпlЛoYП aпoтeЛeí n XopПYnan KUKAo0œo0aui6nç eÎTe ae ev6oфЛéßleQ waeiç, eÎTe a™ tou orôjaToQ. AeûTepn eпlЛoYП aTOTeXouv n aZa9eionpivn aпô tou

orôjaToQ, n KUKAoonopivn aпô tou aTôjaToç25 п то mycophenolate mofetil a™ tou aTôjaToç (xop^

Ynan ei^ôQ eпíanJnQ évôei^nQ).26,27 H aZa9eloпpívn, n

га^атор^н кal to mycophenolate mofetil jrapouv eпíаnQ va xopnYH9oùv ae aa9eveiç je av9eктlкП vôao ara кopтlкoaтepoel6П п aôuvajia eÀâTTwanQ thq ôôanç touq. TéXoç, aa9eveiç je ßapeia кЛlVlкП elкôva rai aпelЛnтlкéQ erönAwaeiQ Yia тп Zw Jпopoûv va uпoßЛn9oûv ae auveôpieç пЛaaJaфaípeanQ.

NOZOZ AAAMANTIAAH-BEHCET

H vôaoç AôajavTiâôn-Behcet eivai jia пoЛuaтnJaтlкП фÀeYJovш6nç vôaoç oyvwothq amoÀoYiaQ пou ioto-ЛoYlкâ xapaктnpíZeтal aпô aYYeiÎTiôa tou пpoaßâЛЛel ôXwv twv jeYe9ûv aYYeia (apTnpieç п фЛéßeQ). Ta ^éov auxvâ пpoaßeßЛnJéva ôpYava eivai to ôépja, oi ßЛevvoYôvol кal oi oф9aÀJoí.28 О eпlпoЛaaJÔQ thq vôaou eivai uфnXôтepoQ атп Méan AvaToX^ тп Meaô-Yeio кal thv Aaia кal n jéan nXiraa eJфâvlanQ eivai Ta 30 éTn. ЛÔYШ thq anJavтlкПQ eTepoYéveiaç thq vôaou кal thq aпouaíaQ eiS^wv Sorajaoiüv Yia тп ôiâYvw-ап thq, éxouv 9eaпlaтeí 6ie9vwç SiaYvwaTira крггп-pia пou пeplЛaJßâvouv eJфâvlan отс^пк^ eÀ^v

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

nivaKaQ 7

IgA ArrEIITIAA

ApaoxiKn ouoia OSOQ Aoon

x°pnvn°nc

XupiQ eKSnXuoeiQ aneiXnTiK£Q yia xn Zwn n

xn Xeixoupyia opyavwv-oroxwv*

npeSviZoXovn pos 30 mg/np.

Me9uXnpe6viZoXovn n pos swq 24 mg/np.

ANGEKTIKH NOZOZ H

AAYNAMIA EAATTQZHZ KOPTIKOZTEPOEIAQN

AZa9eionpivn pos 1-2 mg/kg/np.

Mycophenolate mofetil*** pos 2-3 g/np.

KuKXoonopivn pos 3-5 mg/kg/np.

Me eKSnXuoeiQ aneiXnT'KCQ v[a xn Z^n n xn Xeixoupyia opyavuv-oxoxuv

Me9uXnpeSviZoX6vn npeSviZoAovq

KuKXo^wo^apiSn

AZa9eionpivn Mycophenolate mofetil*** KuKAoonopivn_

iv

pos

KAI ¿va ano Ta napaKaru: 1n eniXoyn

pos iv

pos pos pos

7-15 mg/kg npspn0^ Yia 3 npspsQ 1 mg/kg/np.

1.5-2 mg/kg/np. 15 mg/kg ava 2-3 sp5.

2n eniXoyn

1-2 mg/kg/np.

2-3 g/np. 3-5 mg/kg/np.

ZupnAnpupariKa:

nXaoua0aipeon

* nop0upa, apOpaXyisQ H ap0piTi6a, ve0pm6a je 0uaioXoyiKn AeiToupYia n npwTeivoupia ewQ 0.5-1 gm/24h.

** ve0pm6a je enripeaaMEvri ve0piKn AeiToupYia, npwTeivoupia >1 gm/24h, TaxewQ E^sXiaaoMEvn ansipaMaTOVE0piTi6a (jnvoeiSeiQ axnjaTi-aMoi as >50% twv aneipaMaTwv), vs0pwoiko auvSpojo, aiMoppayia nemiKou, Ku^eAiSiKn aiMoppayia, SYKe0aAiKn aiMoppayia

*** XopHYnan sktoq sntonjnQ evSei^nQ (off-label)

(> 3 0opeQ oe eva etoq) Kai Suo ano Ta aKoAouBa: unoTponiaZovTa eAKn YsvvnTiKwv opYavwv, o0BaAji-kh npoaPoAn je anwAsia TnQ opaanQ, xapaKTHpiaTiKn SepjaTiKH npoaPoAn, BeTiKn SoKijaaia naBepYsiaQ.29 H 9epaneuTiKn npoaeYYian eniAeYSTai je Paan Tn npoaPoAn opYavwv-aToxwv.

OOGAAMIKH nPOZBOAH

Ze nepinTwaeiQ aaBevwv je npoopoAq rou omoOiou OaAayou (onioOia payoeidmda), n Bepaneia auvi-aTaTai aTn xopnYnan auvSuaajou KopTiKoaTspoeiSwv Kai aZaBeionpivnQ (nivaKaQ 8). Ta KopTiKoaTeposiSn xopnYouvTai apxiKa ae evSo0AePieQ Yia 3 njepeQ Kai aKoAouBwQ ano tou aTojaToQ. H aZaBeionpivn xopn-YeiTai ano tou aTojaToQ.30,31 Ze nepirnwan unoTpo-nnQ, xpnaijonoiouvTai Ta BepaneuTiKa axnjaTa TnQ aoPapnQ o09aAjiKnQ npoaPoAnQ, onwQ napaTiBevTai aTn auvexeia.

Ze nepinTwaeiQ ooPapnQ o$9aXMiKhQ npooPoAnQ,

SnAaSn oTav n orniKn o^uTnTa eivai <2/10 ae KAija-Ka 10/10 n/Kai npoaPaAAsTai o aj0iPAnaTpoei6nQ, npoTeiveTai n xopnYnan Infliximab (xopnYnan sktoq enianjnQ evSei^nQ) ev6o0AePiwQ. Eav auTo Sev eivai s0ikto, xpnaijonoieiTai o auvSuaajoQ koptikoots-

poeiSwv Kai aZaBeionpivnQ, onwQ nepiYpa0nKe avw-Tepw. Ze nepinTwaeiQ Suaave^iaQ aTnv aZaBeionpi-vn, jnopei va xpnaijonoinBei KuKAoanopivn ano tou aTojaToQ.32 Oaov a0opa touq avTi-TNF napaYovTSQ, evaAAaKTiKa tou Infliximab jnopei va xpnaijonoinBei Adalimumab (xopnYnan sktoq enianjnQ evSei^nQ).33,34

ArrEIAKH nPOZBOAH

H Bepaneia twv aaBevwv je voao ASajavTia-Sn-Behcet nou ej0aviZouv aYYeiaKn aujjsToxn KaBo-piZsTai ano Tn Bean twv aYYeiwv nou npoaPaAAovTai. Ze nepinTwaei ev ru PaOei QAepoOpoyPuons ruv aKpuv npoTeiveTai n Bepaneia je KopTiKoaTeposiSn ano tou aTojaToQ n evSo0AePiwQ Kai aZaBeionpivn ano tou aTojaToQ (nivaKaQ 9). H nAeov aoPapn aYYeiaKn npoaPoAn nepiAajPavei Tnv ev ru paOei QAepoOpo^puon rns Karu KoiAns QMpaq q ruv nnariKwv QAeptiv (ouvSpoyo Budd-Chiari) Kai rnv eyQavion aprnpiaKwv q nveuyovi-kwv aveupuoyaruv.35 Ztiq nepinTwaeiQ auTeQ, n Bepaneia nepiAajPavei Suo 0aaeiQ: auTn TnQ BepaneiaQ e0oSou Kai auTn TnQ BepaneiaQ auvnipnanQ nou aKo-AouBei. Qq npwTn eniAoYH Yia Tn Bepaneia e0oSou npoTeiveTai n xopnYnan KopTiKoaTspoeiSwv apxiKa ae

nivaKaç 8

0Ф©AЛMIKH ПР01В0ЛН N. AAAMANTIAAH-BEHCET

ApaoTiKh ouoia Oôôç Xophvnonc Aôon

ФЛcyuovh onio9iou 9aAâuou (onio9ia paYosiöiTiöa)

Me9uXnpe6viÇoXôvn npeôviÇoXôvn iv pos h iv Kai pos 7-15 mg/kg/np. Yia 3 npspsc 1 mg/kg/npspa

AZa9eionpivn 2-2.5 mg/kg/npspa

Yn0TP0nH

ВЛ. ©spansia ooßaphc oф9aЛplкhc _пpooßoЛhc_

ApaoTiKh ouoia

Infliximab*

Adalimumab*

npeôviÇoXovn

AÇa9eionpivn

KuKAoonopivn

Oôôç Xophvnonc

Zoßaph o09aAuiKh npooßoAh

(onTiKh oÇÙTnTa < 2/10 os i^ipaKa 10/10) h/Kai пpooßoЛh tou apфlßЛnoтposlöouc)

1n emXoyh

iv

sc

2n eniXoyh

pos h iv

Aôon

5 mg/kg avä 4-8 sßöopäösc 40mg avä 2 sßöopäösc 1 mg/kg/npspa

pos 2-2.5 mg/kg/npspa

h eni ôuoaveÇiaç oTnv aZaOsionpivn

pos_2-5 mg/kg/npspa

* XopÔYnan eкrôQ eraanjnQ évôei^nQ (off-label)

ev6oфЛéßleQ woeiç rai акoЛoû9wQ aпô tou aTôjaToQ п ev6oфЛéßlWQ кal кuкЛoфwaфaJÍ6nQ ae ev6oфÀéßleç jnviaieç waeiç. Qq ôeùTepn eпlЛoYП пpoтeíveтal n xo-

nivaKaç 9

pПYnan Infliximab (xopПYnаn eктÔQ eraanjnQ évôei^nQ) ae auvôuaajô je aC^eio^^ aпô tou orôjaToQ.36 Qq 9epaTOÎa auvтПpnanQ пpoтeíveтal n XopПYnan

ArrEIAKH ПР01В0ЛН N. AAAMANTIAAH-BEHCET

^paçrjKh ouoia npeôviZoAovn AÇa9eionpivn

Aôon

pos pos

©pöußuon KâTU KoiAnç 0AcßaQ h nnaTiKÜv 0Aeßuv ApTnpiaKa h nveuuoviKÙ aveupuouaTa

©EPAnEIA EФ0A0Y

0,5-1 mg/kg/npspa 2-2.5 mg/kg/npspa

Me9uXnpe6viÇoXôvn npeôviÇoXôvn

KuKAo0uo0auî6n

Infliximab* AÇa9eionpivn

1n eniXoyh

iv

pos

Kai ¿va anô Ta napaKârn:

pos

iv

pos

2n emXoyh

7-15 mg/kg np. Yia 3 npspsc 1 mg/kg/npspa

1,5-2 mg/kg/npspa 15 mg/kg avä 2-3 sßö.

5 mg/kg avä 4-8 sßöopäösc

2-2.5 mg/kg/npspa

©EPAnEIA IYNTHPHIHI

AÇa9eionpivn

pos

2-2.5 mg/kg/npspa

* XopÔYnan eкrôQ eraanjnQ évôei^nQ (off-label)

Kai

Kai

Kai

of RHEUMATOLOGY

E ЛЛ H N I К H РЕУМАТОЛОпА

aZa9eloпpívnQ a™ tou aTôjaToç. Qq YevlкП apxП атп 9epaпeía aa9evwv je vôao Aôa-javTiâôn-Behcet кal ev tw ßa9ei фЛeßlкП 9pôJßwan, H ХРпап avтlпnктlкПQ, avтlalJoпeтaЛlaкПQ п avTiivw-soauti^q aYWYПQ avтevôeíкvuтal aфevÔQ xôyw tou XajnXôu кlvôûvou Yia aпoкôЛЛnan tou 9pÔJßou кal eJфâvlan пveuJovlкПQ eJßoÀПç кal aфeтaípou xoyw thq пl9avôтnтaç va auvuпâpxouv aYYelaкâ aveupu-ajâTa, n рп^П twv oпoíwv jrapei va aпoßeí joipaia.35

ПР0ЕВ0ЛИ rAZTPENTEPIK0Y

Ze aa9eveiç je пpoaßoЛП Yaaтpevтeplкoû, oi 9epa-пeuтlкéQ eпlЛoYéQ пeplЛaJßâvouv Ta кopтlкoaтepo-elôП aпô tou aTôjaToç п evôoфЛeßíwQ, aZa9eloпpívn a™ tou aTôjaToç п aouЛфaaaЛaZívn a™ tou aTôjaToç (пívaкaQ 10).

nivaKaç 10

Ze av9eKTiKéç nepinTwoeiç h oe aSuvauia eAdT-Tuonç KopTiKoeiôœv oe Söon <7.5 mg/nuépa,

пpoтeíveтal n ХopПYnan mycophenolate mofetil (xo-pqyqoq sktôç snioq^qç évôsiçqç) апô tou aTôjaToç, га^атор^н aпô tou aTôjaToç п aC^eio^^ aпô tou aTôjaToç. EvaЛЛaктlкâ, Jпopoûv va xpnalJoпol-n9oùv eктÔQ ertanjnç évôei^nQ to infliximab evôoфЛe-ßiwQ п adalimumab uпoôopíwQ.37

ПP0ZB0ЛH AP©PfiZEfiN

Ze ao9eveiç je пpoaßoЛП ap9pшaewv, wq 9epaпeía прщт^ YPaJJПQ пpoтeíveтal n KoAxiKivn38 aпô tou aTôjaToç (пívaкaQ 11). Ze av9eKTiKéç, unoTponid-Zouoeç uop0éQ пpoтeíveтal n ХopПYnan кopтlкoaтe-poelôшv, aZa9eloпpívn aпô tou aTôjaToç п Je9oтpe-^атп a™ tou aTôjaToç п uпoôopíwQ.39 Ze aa9eveiç

ПP0ZB0ЛH rAZTPENTEPIK0Y N. AAAMANTIAAH-BEHCET

ApaoTiKh ouoia Oôôc Xophvnonc Aôon

npeSviZoAövn APXIKH ©EPAnEIA pos h éwc 60 mg/npspa

Me9uXnpe6viZoXövn pos h sa>c 48 mg/npspa

ZouA0aoaAaZivn pos h 3-4 g/npspa

AZa9eionpivn pos 2-2.5 mg/kg/npspa

AN©EKTIKH N0Z0Z H AAYNAMIA EЛATTQZHZ K0PTIK0ZTEP0EIAQN

AZa9eionpivn Mycophenolate mofetil* KuKAoonopivn Infliximab* Adalimumab* pos pos pos iv sc 1-2 mg/kg/npspa 2-3 g/npspa 3-5 mg/kg/npspa 5 mg/kg avä 4-8 sßöopäösc 40mg avä 2 sßöopäösc

* ХopпYnan eicraç eraanjnç évôei^nç (off-label)

nivaKaç 11

ПP0ZB0ЛH AP©PQZEQN N. AAAMANTIAAH-BEHCET

ApaoTiKh ouoia Oôôc Xophvnonc Aôon

KoAxiKÎvn pos 1-2 mg/npspa

AN©EKTIKH N0Z0Z

npeSviZoAövn pos h étt>c 30 mg/npspa

Me9uXnpe6viZoXövn pos h sa>c 24 mg/npspa

AZa9eionpivn pos h 1-2.5 mg/kg/npspa

Me9oTpeÇdTn pos h sc 15-25 mg/sßöopäöa

Av9eKTiKh vöooq ueTd Kai Tn xphon Sùo avoooKaTaoTaATiKwv napayövTuv Kai aSuvauia eAdiruonç Tnç ööonQ tuv KopTiKooTepoeiSwv oe <7.5 mg/nuépa

Infliximab* iv h 5 mg/kg avä 4-8 sßöopäösc

Etanercept* sc h 50 mg/sßöopäöa

Adalimumab* ч sc 40 mg ____n _rï s: ,-f:__

* ХopпYnan e^Q eraanjnç évôei^nç (off-label)

Me avGeKTiKH vóoo meto Kai xn XPH^H Súo avooo-KaxaoxaAxiKuv napayóvrov Kai aSuvapía eAáx-xwonQ xnQ SóonQ xwv KopxiKoorepoeiSúv oe <7.5

mg/nMÉpa, npoTeíveTai n eKTóQ eníonpnQ évSei^nQ XopnYnan infliximab evSo0AePíwQ n adalimumab unoSopíwQ n etanercept unoSopíwQ.40

nPOZBOAH KENTPIKOY NEYPIKOY ZYZTHMATOZ (NEURO-BEHCET)

H npooPoAn tou KevTpiKoú veupiKoú ouoTnpaToQ oTn vóoo ASapavTiáSn-Behcet pnopeí va AáPei napeYxu-paTiKñ Kai pn napeYxupaTiwi pop<t>n,41 pe Tn SeúTepn va a0opá Tn 9pópPwon 0AePwv n onpaYYwSwv KóA-nwv (nívaKaQ 12).

ztti napeyxuMaxiKn Mop$ñ, npoTeíveTai n xopnYnon ouvSuaopoú nou nepiAapPávei KopTiKooTepoeiSn Kai avoooKaTaoTaATiKñ/avoooTpononoinTiKñ 9epaneía. Ta KopTiKooTepoeiSn xopnYoúvTai wq evSo0AéPieQ woeiQ Yia 3 npépeQ Kai aKoAoú9wQ xopnYnon anó tou oTópaToQ n evSo0AePíwQ. EninAéov twv KopTiKooTepo-eiSwv, wq npwTn eniAoYñ npoTeíveTai n xopnYnon ku-KAo0wo0apíS|Q oe evSo0AéPieQ pnviaíeQ woeiQ. EvaA-AaKTiKá wq SeúTepn eniAoYñ, npoTeíveTai n xopnYnon aZa9eionpívnQ anó tou oTópaToQ n pe9oTpe;áTnQ anó tou oTópaToQ n unoSopíwQ. Ze av0eKriK¿Q ne-pinxúoeiQ n oe unoxponn unó 0epaneía ouvxnpn-onQ, npoTeíveTai n eKTóQ eníonpnQ évSei^nQ xopnYnon infliximab evSo0AePíwQ n adalimumab unoSopíwQ.42

Mn napeyxuMaxiKn Mop$ñ, npoTeíveTai n xopn-Ynon KopTiKooTepoeiSwv anó tou oTópaToQ n evSo-0AePíwQ. H xpnon KuKAoonopívnQ avTevSeíKvuTai ot|v npooPoAn tou KN! Aóyw veupoTo;iKóT|TaQ, eKTóQ av ouvunápxei o09aApiKn npooPoAn Kai avTévSei^n ot| xpnon twv unóAoinwv 9epaneuTiKwv eniAoYwv.35

nPOZBOAH AEPMATOZ - BAENNOrONQN

H npooPoAn tou SéppaToQ Kai twv PAevvoYóvwv ot| vóoo ASapavTiáSn-Behcet eívai e^aipeTiKá ouxvn Kai pnopeí va AáPei ti pop0n twv pepovwpévwv eAKwv oTópaToQ Kai YevvnTiKwv opYávwv, aKpópop0ou e;av9npaToQ n oZwSouQ epu9npaToQ (nívaKaQ 13). népav tiq e0appoYnQ ToniKnQ 9epaneíaQ, npoTeíveTai n xopnYnon KoAxiKívnQ38 anó tou oTópaToQ n Kop-TiKooTepoeiSwv. av9eKTiKéQ nepinTwoeiQ, npoTeíveTai n xopnYnon aZa9eionpívnQ anó tou oTópaToQ, KuKAoonopívnQ anó tou oTópaToQ n pe9oTpe;áT|Q anó tou oTópaToQ n unoSopíwQ. Ze ao0eveíQ me av0eKTiKn vóoo Mexá Kai xn xpn-on aZa0eionpívnQ Kai KoAxiKívnQ Kai aSuvapía eAáxxwonQ xnQ SóonQ twv KopxiKooxepoeiSwv oe <7.5 mg/nMÉpa, npoTeíveTai n eKTóQ eníonpnQ évSei-;nQ xopnYnon infliximab n adalimumab unoSopíwQ n etanercept unoSopíwQ.40,43

KPYOZOAIPINAIMIKH ArrEIITIAA

Qq Kpuoo$aipíveQ opíZovTai oi avoooo0aipíveQ nou Ka9iZávouv in vitro oe 9eppoKpaoíeQ piKpóTepeQ twv 37oC Kai SiaAúovTai óTav enava9eppav9oúv. Kpuo-o^aipivaiMÍa eívai n napouoía Kpuoo0aipivwv otov opó Kai SiaKpíveTai oe:

■ AnÁq KpuooQaipivaiiJÍa (TÚnou I): SnpioupYeíTai anó povoKAwviKéQ avoooo^aipíveQ evóQ iooTúnou (ou-vn9wQ IgM, onavióTepa IgG, IgA, eAa0péQ aAúoeiQ)

■ MsiKrq KpuooQaipivaiiJÍa (TÚnou II Kai TÚnou III): SnpioupYeíTai anó Súo n nepiooóTepwv iooTúnwv avo-ooo0aipíveQ, eíTe povoKAwviKéQ (TÚnou II) eíTe noAu-KAwviKéQ (TÚnou III). Oi Kpuoo0aipíveQ auTéQ éxouv SpaoTnpióT|Ta peupaToeiSoúQ napáYovTa (RF) Kai eívai ouvn9wQ IgM.

nívaKaQ 12

nPOZBOAH KNZ N. AAAMANTÍAAH-BEHCET

ApaariKñ ouaía OSóc Xopñvnons Aóan

napeyxuMaxiKn Mop$ñ

Me0uAnpeSviZoAóvn iv 7-15 mg/kg/nu.Yia 3 nuspsc

npeSviZoAóvn pos KAI eva anó ra napaKáru: 1n emAoyn 1 mg/kg/nuspa

KuKAo^uo^apíSn iv 2n eniAoyñ 15 mg/kg avá 2-3 sp5.

AZa0eionpívn pos 2-2.5 mg/kg/nuspa

Me0oxpe^áxn pos n sc 15-25 mg/sP5ouá5a

AN0EKTIKH NOZOZ H YnOTPOnH YnO ©EPAnEIA ZYNTHPHZHZ

Infliximab* iv q svaÁAaKriKá 5 mg/kg avá 4-8 £p5ouá5sc

Adalimumab* sc 40 mg avá 2 £P5ouá5sc

Mn napeyxuMaxiKn Mop^n (GpóuPmon $AsPwv n onpaYYtt>5wv KÓAnoiv)

npsóviZoAóvn pos étt>C 60 mg/nuspa

MsGuAnDsóviCoAóvn pos éttic 48 mg/nuspa

* XopnYnon eKTóQ eníonpnQ évSei^nQ (off-label)

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

nivaKaç 13

nPQIBQAH AEPMATOZ-BAENNOrONQN N. AAAMANTIAAH-BEHCET

ApaoTiKh ouoia

Oôôç Xophvnonc

Aôon

Me^ovw^eva cAkh oxopaToq Kai ycvvnxiKüv opyävwv Kai aK^o^opQo cÇâvSnMa

OÇwôeç cpüSnMa

KoAx'KÎvn npeôviÇoXovn McGuAnpcôviÇoAôv^

ApaoriKh ouoia

AÇa9eionpivn KuKAoonopivn

Me9oTpefàTn

pos

pos

pos

AN0EKTIKH NOIOI

Oôôç Xophvnonc

pos pos pos n sc

1-2 rng/r||J£pa éoiç 30 mg/r||Jspa

éwç 24m||ji®pa

Aôon

1-2.5 mg/kg/r||Jspa

2-5 mg/kg/r||Jspa 15-25 mg/sßöouciöa

le ao9eveiç m£ av9cKTiKn vooo peTà Kai Tn XPH°n aÇa9eionpivnç Kai KoAxiKÎvnç Kai aSuva^îa eAâTTwonç Tnç Soonç tuv

KopTiKooTepoeiSwv oe <7.5 ma/nuépa

ApaoriKh ouoia

Infliximab*

Adalimumab*

Etanercept*

Oôôç Xophvnonc

iv

h

sc

h

sc

Aôon

5 mg/kg ava 4-8 sßöo|jciösQ

40 mg ava 2 sßöo|j<CösQ _50 mg/sßöouaöa_

* XopnYnon ektoç enianpnç évôei^nç (off-label)

0 ôpoQ Kpuoo^aipivaiMiKn aweiÎTiôa a0opâ pia aYYeiÎTiôa piKpwv aYYeiwv pe npoaßoAn noAAanAwv opyâvwv nou o0eiAeTai aTnv evanô9ean Kpuoa0aipi-vwv aTo Toixwpa twv aYYeiwv. To 0âapa twv eKôn-Awaewv Tnç eivai eupù Kai KupaiveTai anô nnia (n.x. nepioôiKn nop0ûpa) péxpi Kai npoaßoAn aneiAnTiKn Yia Tn Zwn.44

H eniÀoYn Tnç 9epaneiaç ae aa9eveiç pe Kpuoa0aipi-vaipiKn aYYeiÎTiôa ßaaiZeTai aTn ßapüTnTa Tnç vôaou. fiç hnia vôooç opiZeTai n napouaia nepioôiraiç nop-0ûpaç, ap9paAYiwv/ap9pÎTiôaç, YeviKwv aupmwpâ-twv, nniaç veuponâ9eiaç, nniaç aneipapaTove^piTi-ôaç (0uaioÀoYiKn KpeaTivivn, anouaia aoßapnç Aeu-KwpaToupiaç).

ZTn ooßaphlyEviKEu^Evn vôoo KaTaTâaaovTai ôÀoi

01 unôÀoinoi aa9eveiç, nÀnv auTwv nou nepiYpâ<t>o-vTai aTnv nepiopiapévn vôao, ônAaôn aa9eveiç pe ôeppaTiKâ éÀKn n iaxaipia, aoßapn veuponâ9eia, aneipapaTove^piTiôa pe ve0piKn avenâpKeia n ve-0pwaiKô aùvôpopo Kai n auppeToxn tou YaaTpevTe-piKoù auaTnpaToç.

^Tnv aneiÀnriKh yia rn Zwq vôao KaTaTâaaovTai aa9eveiç pe Taxéwç e^eAiaaôpevn aneipapaTove^pi-Tiôa, auppeToxn tou KNI, peaevTépio iaxaipia Kai ku-^eÀiôiKn aipoppaYÎa.

rENIKEZ APXEZ OEPAnEIAZ

ApxiKq avoooKamoraATiKq Qspansia: AvoaoKaTa-aTaÀTiKn 9epaneia npénei va ôiveTai apxiKâ ae aa9e-veiç pe kàivikéç eKônAwaeiç Kpuoa^aipivaipiKnç aYYei-ÎTiôaç, ave^âpTnTa anô to unoKeipevo aÎTio. H 9epa-

neia auviaTaTai ae xopnYnan KopTiKoaTepoeiôwv ae auvôuaapô n ôxi pe rituximab n KuKÀo0wa0api5n Kai, ae Kânoiouç aa9eveiç, nAaapa^aipean. Qspansia tou unoKsiysvou aiTÎou: OAoi oi aa9eveiç npénei va Aapßävouv 9epaneia nou va KaTeu9ùveTai aTo unoKeipevo aÎTio. ria napâôeiYpa aa9eveiç pe xpôvia nnaTÎTiôa C o0eiAouv va Aäßouv avTiiKn aYW-Yn, evw aa9eveiç pe unoKeipevo Aep0oûnepnAaaTiKô vôanpa o0eiÀouv va Aäßouv avTÎaToixn 9epaneia.

OEPAnEIA KPYOZOAIPINAIMIKHZ ArrEIITIAAZ (TYnOZ II/III) MH ZXETIZOMENHZ ME HCV AOIMQEH

'Hnia voooç: (nop0ûpa, ap9paAYÎeç, nnia noAuveu-ponâ9eia, aneipapaTove^piTiôa xwpiç ennpeaapô Tnç ve0piKnç AeiToupYÎaç)

npeôviÇoAôvn (n avâAoYn ôôan pe9uAnpeôviZoAô-vnç) anô tou aTôpaToç. OTav eniTeux9ei éAeYXoç Tnç vôaou npénei va YÎvei aTaôiaKn peiwan Tnç ôôanç twv KopTiKoaTepoeiôwv Kai va ôiaKonoûv ôTav auTô eivai ôuvaTô.

McTpia/Zoßapn voooç: (AeppaTiKâ éAKn, aia9n-TiKoKivnTiKn veuponâ9eia, aneipapaTove^piTiôa pe ve0piKn avenâpKeia n/Kai anpavTiKn AeuKwpaToupia, npoaßoAn nveupôvwv, YaaTpevTepiKoù, KNZ). ria Touç aa9eveiç pe péTpia npoç aoßapn vôao n 9e-paneia 9a npénei va nepiAapßävei KopTiKoaTepoeiôn ae auvôuaapô pe rituximab n KuKAo0wa0apiôn45 (nivaKaç 14). AvaAuTiKôTepa, apxiKâ xopnYeÎTai pe9uA-npeôviZoAôvn ae evöo<i^ß^ waeiç Yia 3 npépeç Kai aKoAoù9wç xopnYeÎTai npeôviZoAôvn ae ôôan ôxi

peYaЛúтepп aпó 80 mg/пpépa. Ze ao6eveíQ pe ппю-тepоu ßa9pоú e^Awoeiq aпó auтéQ поu проava-фépоvтal pпоpоúv va пapaЛelф9оúv о1 evбофЛéßleQ woeiç коpтlкоoтepоelбшv.

EпíoпQ, eпlЛоYП прштпс YPappПQ Yia тп ouvбuaoтlкП 9epaпeía eívai п evбофЛéßla xоpПYПoп Rituximab.46 H ouYкeкplpévп 9epапeíа xоpпYeíтal eKróQ eпíoпpПQ évбel^ПQ тоu oKeuáopaTOQ.

EvaЛЛaкгlкП eпlЛоYП прштпс YPappПQ eívai п к^ кAoфwoфauíбп, п craía pпоpeí va xоpПYП9eí oe evбофÀéßleç woeiç п aпó тоu orépaTOQ. H бóoп тпд кuкЛофшoфapíбпQ тропопоюпги avaÀÔYWç тпд п^-KíaQ, тпс veфрlкПQ ЛelтоuрYÍaQ Kai тоu api9pcú тшv ÁeuKúv, 0пшд пeрlYрáфeтal oto oxenKó пaрáртпpa. Kaтá тпv aрxlкП 9eрaпeía Yia тпv крuоoфalрlvalplкП aYYeinrôa Kai oe ao9eveíQ поu пЛпроúv ouYKeKpi-péveç evбeÍ^elQ, ouvloтáтal п évaр^п пЛaopaфaÍрe-oпQ.47 Oi evбeÍ^elQ auтéQ eívai:

- toxéwq e^eЛlooópevп oпelрapaтоveфрÍтlбa (Kpean-vívп орои > 5.6 mg/dl)

- ra^eA^iKí aipcppaYÍa

- eкгeтapéva бeрpaтlкá еЛкп п YáYYPaiva áKpwv

- av9eкгlкП п прообeuтlкá e^;eЛюoópevп пeрlфeрlкП veuропá9ela

- кeрauvоßóÀоç поЛuорYavlкП ouppeтоxП.

H бóoп тпс пЛaopaфaÍрeoпQ eívai 1 - 1.5 форá тоu оЛlкоú óykcu пЛáopaтоQ (TPV) avá ouveбрÍa Kai п

ouxvóтптá тпс pía ouveбрÍa пpeрпoÍwç п pépa пaр' пpéрa (avaÁóYWQ évбel^ПQ). H пЛaopaфaÍрeoп пре-пel va YÍveтal oe eÇetôiKeupéva vоoпЛeuпкá кévтрa pe epпelрía o^v eфaрpоYП тпд pe9óбоu. ZupпЛпршpaтlкá, о1 ao9eveÍQ поu Лapßávоuv aYW-YП pe кuкЛофшoфapÍбп п Rituximab ouvютáтаl va Лapßávоuv xПPelопрофúЛa^п évavn Pneumocystis jirovecii pe Tрlpe9опрÍpп/oоuЛфape9о^aZóЛп aпó tou oropaTOQ Ka9úQ Kai avтюoтeопорwтlкП aYWY^

YпoтpoпláZouoa vóooq

Oi eÁáoocveQ шотропед avтlpeтшпÍZоvтal pe тро-попоÍпoп тпд бóoпQ тшv пбп Aapßavcpevwv корп-коoтeроelбшv, xPП0lpопоlwvтaç то oxПpa пои xорп-Yeíтal oтпv Ппla vóoc. Oi peíZpveQ uпотропéQ avn-peтwпÍZоvтal pe тa oxПpaтa поц> протeÍvоvтal oтп 9eрaпeía тпд oоßaрПQ vóocu.

OEPAnEIA KPYOIФAIPINAIMIKИI AГГEIITIЛAZ IXETIZOMENHI ME HCV ЛOIMQ=И

Zтп 9eрaпeía тпд pelкгПQ крuоoфalрlvalplкПQ aYYeií-тlбaQ (типоц) II/III) поц> oxeтÍZeтal pe xрóvra ппaтíтlбa C, a^cY^viafo Aí9c o^v avnpe'1'шпюп aпотeЛeÍ п кaтaпоЛépпoп тпд xрóvlaQ HCV Acípu^Q pe avrii^ вepaпeía.48,49 H avтllкП 9eрaпeía прéпel va бíveтal пávтa oe ouveрYaoÍa pe e^e^iKeupevc ппaтоЛóYо, wore va aпофaoютeÍ epпeрютaтшpéva то eÍбоQ Kai

nívaraq 14

KPYOIФAIPINAIMIKИ ArrEIITIAA (MH IXETIZOMENH ME HCV ЛOIMQHИ)

ApaaTiKñ ouaía 00óq XopñvnanS Aóan

HnIA NOIOI*

ПpeбvlZoAóvn pos éwc 30 mg/nuspa

MeвuAпpeбvlZoAóvn П pos éwc 24 mg/nuspa

METPIA-IOBAPH NOIOI**

MeвuAпpeбvlZoAóvn ПpeбvlZoAóvn iv pos Kai 1n emAovn iv pos iv 7-15 mg/kg nuspnoi^c Yia 3 nuspsc 0,5-1 mg/kg/nuspa

Rituximab*** KuкAoфыoфаuíбn 375 mg/m2 avá sp6ouá6a Yia 4 sp6ouá6sc П 1 gm Kai sпaváЛnФn usTá 2 sp6ouá6sc 1,5-2 mg/kg/nuspa П 15 mg/kg avá 2-3 sP6ouá6sc

Eáv ouvuпápxel cva aпó то катывк

1. Taxéuç e^eÁiooójevq oпеlрaJaтоvефрíтlбa

2. КифеЛб1Kq aijoppaYÍa

3. EKTemjéva 6spjaTiKá éÁKq q YáYYPaiva áKpwv

4. AvQsKTiKq / прообеитiKq перферiKq vеuропáвеla

5.Kspauvoß0Xoq поÁuорYavlкq oujjemxq

_Tóte, emnÁeov:_

_nAaoui^aípeon_

* Порфúрa, aр9рaЛYÍeç/aр9рÍтlбa, YeviKá oupmupaia, ппга oпelрapaтcveфрÍтlбa: фuoюÀCYlкп ^ea™^ Kai x^îç ocßaрп Лeuкшpaтcuрía: < 0.5-1 gm/24h, ппга пcÀuveuрcпá9ela

** Лeрpaтlкá еЛгал, aю9п"пкcкlvптжп veuрcпá9ela, oоßaрп oпelрapaтcveфрÍтlбa, прcoßcЛп ™eupóvwv, Yaoтрevтeрlкcú, KNZ *** XcрпYП0П eicroç eпÍ0ПPПQ évбe^пç (off-label)

of RHEUMATOLOGY 2

E Л Л H N I К H РЕУМАТОЛОПА 2015

П ôiâpKeia Tnç avTiiKiç aYwYiç, Ka9wç Kai п парако-Aoû9non тои ao9evoûç.

Ze ao9eveiç пои éxouv ooPapéç KAiviKéç eKônAwoeiç

anô Tnv криоофа1рма1|жп aYYeiÎTiôa Kai 9a AâPouv

apxiKii 9epaneia pe avoooKaTaoTaÀTiKoûQ napâYo-

vTeç eivai onMavTiKô n avTiiKi 9epaneia pe oxipaTa

nou nepiAapPâvouv lvтepфepôvn va Ka9uoTepei Yia

ôiâoTnpa anô évav wç Téooepiç piveç. H Aoyik| au-

t|ç Tnç Ka9uoTépnonç eivai:

(a) n avoooKaTaoTaÀTiKi 9epaneia pnopei va PeA-

Tiwoei TaxûTepa тп фAeYpovi Kai тп PAâPn oe ôpYa-

va-oTÔxouç, evw n KAiviKi PeATÎwon anô Tnv avTiiKi

9epaneia avapéveTai va eivai nio apYi

(P) n xopiYnon lvтeрфeрôvnç pnopei va oônYioei oe

e^âpoeiç Tnç кpuooфalplvalpíaç, Tnç aYYeiÎTiôaç i âA-

Awv auToâvoowv eKônAwoewv

(Y) n aпoфuYП twv napevepYeiwv anô to ouvôuaopô

twv ôûo 9epaneiwv

(ô) n avTiiKi aYWYi pnopei va npoKaAéoei veфplкéç, veupoAoYiKéç i ôeppaTiKéç eKônAwoeiç nou va ouy-xéovTai pe pn avTanôKpion Tnç кpuooфapvalplкiç aYYeiÎTiôaç oTnv avoooKaTaoTaATiKi 9epaneia. Méxpi oipepa ôev éxei a£ioAoYn9ei n anoTeAeopaTi-KôTnTa, n aoфâAela Kai o KaTaAAnAAôTepoç xpôvoç xopÎYnonç twv veoTépwv aneu9eiaç ôpwvTwv avTi-ikwv фappâкwv (Direct Acting Antivirals, DAAs) ano tou oTôpaToç oe auTi Tnv opâôa ao9evwv.50,51 Ooov aфopâ Tnv avoGOKaxaCTxaAxiKn Gepaneia aKoAou9oûvTai oi iôieç oônYÎeç pe Tn 9epaneia Tnç peiKTÎç кpuooфalplvalplкпç aYYeiÎTiôaç nou ôe oxeTÎ-ZeTai pe HCV Aoipw^n, ônwç пeplYpâфnкav napanâ-vw (nivaKaç 14). Ze auTiv Tnv KaTnYopia twv ao9e-vwv, oTaôiaKâ ouYKevTpwvovrai apKeTâ ôeôopéva Yia Tnv eniTuxi avTipeTwnion pe xopiYnon rituximab.52,53

OZQAHZ ПOЛYAPTHPITIЛA

H oZwônç noAuapTnpÎTiôa (Polyarteritis nodosa, PAN) eivai pia ouoTnpaTiKi veKpwTiKi aYYeiÎTiôa nou npo-oPâAAei Kupiwç apTnpieç péoou peYé9ouç xwpiç na-pouoia oпelpapaтoveфpíтlôaç Kai nou ôe ouoxeTÎZe-Tai pe ANCA avTiowpaTa.54 H 9epaneuTiKi npooéYYion oTnv PAN e^apTâTai anô Tiç aKôAou9eç peTaPAnTéç, oi onoieç npénei va Ka9o-piZovTai npiv anô Tnv évap^n Tnç 9epaneiaç:55

- Tn ooPapôTnTa Tnç vôoou (npooPoAi)

- Tn napouoia i pn ioYevoûç nnaTÎTiôaç

- Tn napouoia pepovwpévnç ôeppaTiKiç i npooPo-Aiç evôç opYâvou anô Tn vôoo.

Tpeiç popфéç Tnç vôoou éxouv пeplYpaфeí. AuTéç eivai: (a) n iôiona9iç popфi, (P) PAN oxeTiZôpevn pe ioYevi nnaTÎTiôa (Kupiwç HBV), Kai (y) n pepovwpévn ôeppaTiKi.

H PapÛTnTa Tnç npooPoAiç eivai onpavTiKôç napâYo-vTaç Yia Tnv eniAoYi Tnç 9epaneiaç: H qnia vôooç nepiAapPâvei ao9eveiç pe YeviKâ ou-

pnTwpaTa, ap9pÎTiôa, avaipia, ôeppaTiKéç PAâPeç, aAAâ фuoloAoYlкп veфplкi AeiToupYÎa Kai anouoia on-pavTiKÎç KapôiaKÎç, veupoAoYiKiç, YaoTpevTepiKiç i âAAnç npooPoAiç.

H ¡jsTpia npoç oofiapq vôooç nepiAapPâvei ao9eveiç pe onoioôinoTe Pa9pô veфplкiç avenâpKeiaç, véa i eniôeivwpévn apTnpiaKi unépTaon oфelAôpevn oTnv aYYeiÎTiôa, oupmwpaTiKi apTnpiaKi oTévwon, aveu-pûopaTa, onoiaôinoTe ioxaipiKi vôoo (n.x. âKpwv, KapôiaKi, YaoTpevTepiKoù, veupoAoYiKÎ).56

1ЛЮПА0Н!

Ze ao9eveiç nou ôev avaKaAûnTeTai unoKeipevo aÎTio ni9avwç oxeTiZôpevo pe Tnv PAN éxoupe Tiç napaKâ-tw 9epaneuTiKéç eniAoYéç avaAôYwç pe Tn PapÛTnTa (nivaKaç 15).

'Hnia vôooç

H 9epaneia ao9evwv pe inia vôoo nepiAapPâvei Tn xopÎYnon npeôviZoAôvnç (péYioTn ôôon 60-80mg/ npépa). H apxiKÎ ôôon 9a npénei va ouvexioTei Yia 4 epôopâôeç, onôTe Kai 9a npénei va YÎvei oTaôiaKi peiwon Tnç ôôonç av unâpxei PeATÎwon (unoxwpnon Tnç ap9pÎTiôaç/ap9paAYÎaç, twv YeviKwv oupnTwpâ-twv, twv ôeppaTiKwv pAapWv Kai pn epфâvlon véwv eKônAwoewv Tnç vôoou). H oTaôiaKi peiwon Tnç ôôonç Tnç npeôviZoAôvnç npénei va YÎvei éToi woTe otov 3o - 4o piva va фтâoel Ta 20 mg/npépa pe nepaiTé-pw oтaôlaкi peiwon Tnç ôôonç Kai otôxo n ouvoAiKi ôiâpKeia Tnç xopiYnonç KopTiKooTepoeiôwv va pnv unepPaivei Touç 6-8 piveç.57

Ze ao9eveiç pe vôoo av9eKTiKi oTa KopTiKooTepoei-ôi i oe aôuvapia peiwonç Tnç ôôonç twv koptiko-oTepoeiôwv oe aveKTâ/anoôeKTâ enineôa, xopnYeÎTai eninpôo9eTn avoooKaTaoTaATiKi aYwYi.57,58 npwTn eniAoYi eivai n aZaGeionpivn i n MeGoxpeÇàxn anô tou oTôpaToç i unoôopiwç. H ôiâpKeia Tnç aYwYiç eivai Yia ¿va xpôvo TouAâxioTov peTâ Tnv enÎTeu^n ûфeonç. H eniAoYi peTa^û pe9oTpe£âTnç Kai aZa9ei-onpivnç npénei va YÎveTai pe pâon to e^aTopiKeupévo пpoфíA To^iKôTnTaç Yia Kâ9e ao9evi Kai Tiç npoTipi-oeiç tou ao9evoûç Kai tou 9epânovTa. AeÛTepn eniAoYi eivai n xopiYnon mycophenolate mofetil (9epaneia eKTôç enionpnç évôei^nç).

MéTpia - ooPapi vôooç

H 9epaneia twv ao9evwv nepiAapPâvei ôûo фâoelç: Tnv apxiKi 9epaneia eфôôou/eпaYWYiç Kai Tn 9epa-neia ouvTipnonç.

H npwTn фâon nepiAapPâvei woeiç MeGuAnpeôvi-ZoAôvnç Yia 3 npépeç), Kai aKoAoû9wç xopiYnon npeôviZoAôvnç anô tou oTôpaToç i evôoфAePíwç pe péYioTn ôôon Ta 60 - 80 mg/npépa. ria Tn oTaôiaKi eAâTTwon Tnç ôôonç twv KopTiKooTepoeiôwv, ioxûei ô,ti Kai oTnv inia vôoo. ZTn фâon auTi, n xopiYnon

тшv кортlкоoтeроelбшv ouvбuáZeтal pe кuкAoфw-oфauíбп.59,60

Ze торпш0п vó00u av9eкгlкПc oто ouvбuaopó кор-тlкоoтeроelбшv Kai кuкÀофwoфapÍбпc п av uпáрxоuv avтevбeí^elc oтп xорПYП0П тпc кuкÀофwoфapÍбпc, pпороúv va xPП0lpопоlп9оúv úoeic evбофÀéßlwv кортlкоoтeроelбшv ^wc пeрlYрáфпкav пaрaпávw) Kai ra™i0c avоoокaтaoтaЛтlкóc пaрáYоvтac поu бev éxei ;avaxрпolpопоlп9eÍ 0то ouYкeкрlpévо ao9e-vП (aZ^era^^, pe9отрe;áтп, MMF) 0тп péYloтп aveкгП б00п.

H бeúтeрп фáoп пeрlЛapßável тп 9eрaпeía ouv^^ oпc. Qc YevlкП aрxП, П 9eрaпeía ouvтПрпoпc прéпel va бгарш тоuЛáxloтоv 18-24 pПvec peтá ^v eпíтeu-;п úфeoпc. Прштп eпlЛоYП eívai п aZaвeloпpívп aпó tou oroparoc п П мeвoтpeÇáтп ап0 тоu oтópaтоc п uпоборÍшc. Лeúтeрп eпlЛоYП aпотeЛeÍ п X0рПYП0П

mycophenolate mofetil (MMF). ^н^пр^мот^ aYWYП

O eпaркПc éÀeYX0c тпc aртпрlaкПc пÍeoпc oтпv PAN eívai oпpavтlкóc, i6íwc oe ouvúпaр;п veфрlкПc ave-пáркerac. ПротeÍveтal п X0рПYП0П evóc avaoroAéa тou ueтaтpeптlкoú evZúuou (aMEA) Ka9úc eívai lбlaíтeрa aпотeЛeopaтlкоÍ oe éбaфоc veфрlкПc ave-пáркerac-ЛeuкwpaтоuрÍac. Yпáрxel W0ró00 о kív6u-v0c eпlбeÍvшoпc ^c veфрlкПc aveпáркelac, aóyw тпc áрoпc тпc eпÍбрaoпc тпc aYYelотevoÍvпc II 0тп бга-

тпрп0п тпc o^^pan^c бlП9пoпc. Eroi, av п XPП-0П aMEA прокaЛéoel oпpavтlкП а^П0П (>30%) oтпv крeaтlvívп ороú, 9a прéпel va xPП0lpопоlп9eÍ avriu-пepтaolкП aYWYП áAAnQ кaтпYopíaç (пx. avaoro-Aeíc бraúÀwv aoßeoтÍоu).

ZupпЛпршpaтlкá, о1 ao9eveíc поu Лapßávоuv aYW-YП pe кuкЛофшoфapÍбп п Rituximab ouvютáтаl va Лapßávоuv xПPelопрофúЛa^п évavтl Pneumocystis jirovecii pe Tрlpe9опрÍpп/oоuЛфape9о^aZóЛп aпó tou oropaTOc Ka9úc Kai avтюoтeопорwтlкП aYWY^

IXETIZOMENH ME XPONIA HBV AOIMQEH

Hпla vó00c

Ze Ппla vó00 pпороúpe va xPП0lpопоlпoоúpe avтllкП aYœYП ^c pоvо9eрaпeÍa. H avтllкП aYWYП прéпel va бíveтal пávтa oe ouveрYaoÍa pe e^e^iKeupevc ппа-toáóy0, шoтe va aпофaoloтeÍ epпeрютaтwpéva то eÍбоc Kai п бláркela mic avтllкПc aYШYПc, Ka9wc Kai п пaрaкоЛоú9пoп тоu ao9ev0úc. Протlpоúvтal та av^-iKá фáрpaкa aпó rou oroparoc (entecavir, tenofovir) поu epфаvÍZоuv plкрП пl9avóтптa epфávloпc avrï-oтaoпc oe paкроxрóvla X0рПYП0П п П пeYKuЛlшpévп lvтeрфeрóvп a.61

Méтрla - oоßaрП vó00c

Ao9eveíc поu epфavÍZоvтal pe oоßaрéc e^Awoeic 0zw60uc поЛuaртпрlÍтlбac 9a прéпel va аут^ти™-Z0vroi pe ouvбuaopó avтllкПc Kai avоoокaтaoтaЛтl-кПc aYШYПc. ria тпv аутикп aYШYП ioxúei 0,т1 Kai o^v Ппla vó00.

Oi ao9eveíc pe oоßaрéc e^Awoeic pпороúv va шфeЛп9оúv ап0 тп X0рПYП0П avоoокaтaoтaЛтlкПc aYWYПc ßрaxeíac бláркelac, бÍvоvтac XPÓvо o^v avтllкП aYШYП va брáoel aпотeЛeopaтlкá oтпv ката-поЛépпoп тоu an"fou, бпАабп тпc XPÓvlac HBV A0Ípw-Éfic. T0 eÍбоc тпc avоoокaтaoтaЛтlкПc aYШYПc eívai пaрópою pe auro поu пeрlYрáфпкe oтпv lбюпa9П PорфП.

AEPMATIKH OZ^HI ПOЛYAPTИPIITIЛA

H 9eрaпeuтlкП проoéYYl0П тпc pepоvшpévпc PAN п PAN evóc орYávоu e;aртáтal ап0 то eÍбоc тоu l0T0Ú tou проoßáЛЛeтal. H бeрpaтlкП PAN YeviKá avтlpeтшпÍZeтal пaрópоla pe тпv ппга ouoтпpaтlкП PAN, evú п проoßоÀП evóc póv0 орYávоu pпорeÍ va aкоÀоu9Пoel pоvокuкЛlкП порeÍa. Zтпv ^eicvó^a тшv пeрlптшoeшv п 9eрaпeía пЛпv тпc xelроuрYlкПc eкгоpПc (п.x. PAN xоAпбóxоu кúoтпc) бev eívai апа-рaíтптп Kai aркоúpaoтe oтпv пaрaкоЛоú9пoп тшv ao9evúv, avá трÍpпvо Yia rov пршто xрóvо Kai avá 6 pe 12 pПvec 0тп ouvéxeia.62 0eрaпeuтlкéc eпlЛоYéc oтпv avтlpeтwпюп тпc бeр-paтlкПc 0ZW60UC поЛuaртпрlÍтlбac поu 9eшрeíтal Ппla oe ßaрúтптa Kai екга0п aпотeЛоúv та pп oтeрl-vоelбП avтlфЛeYpоvшбп фáрpaкa, п коЛxlкÍvп, та кор-тlкоoтeроelбП ап0 тоu oтópaтоc (~0.5 mg/kg/пpéрa) Kai áAAa фáрpaкa (бaфóvп, uбро£;uxAшрокÍvп). Ze pп avтaпóкрloп, pпорeÍ va xорпYП9оúv au^pévec 6ó-oeic кортlкоelбшv. ria тп 0табгакп peÍшoп тпc бóoпc тшv кортlкоoтeроelбшv ioxúei 0,т1 Kai o^v Ппla vó00 oтпv йюпа9п о^шбп поЛuaртпрÍтlбa. Ze ao9eveíc pe vó00 av9eкгlкП 0та кортlкоoтeроelбП п oe aбuvapía peÍшoпc тпc бóoпc тшv кортlкоoтeро-etôwv oe aveктá/aпобeктá eпíпeбa, X0рпY0úvтal era-прóo9eтa aZaвeloпpívп п мeвoтpeÇáтп óпшc éxei пeрlYрaфeÍ пaрaпávш. Zпávla Kai oe toAú oоßaрéc п av9eKnKec пeрlптшoelc 9a pпороúoe va xPП0lpопоl-П9eí Kai п кuкAофwoфapÍбп pe ßáoп та oxПpaтa поu éx0uv пбп avaфeр9eí.

mediterranean journal 26

of RHEUMATOLOGY 2

E Л Л H N 1 К H РЕУМАТОЛОГ1А 2015

nivaKaç 15

IAI0nA©HZ 0ZQAHZ П0ЛYAPTHPITIДA

ApaoTiKh ouoia Oôôc Xophvnonc Aôon

HnIA N0Z0Z

npeSviZoAövn pos h 40-60 mg/npspa

Me9uAnpe6viZoAövn pos 32-48 mg/npspa

METPIA/Z0BAPH N0Z0Z

©EPAnEIA EФ0A0Y

Me9uAnpe6viZoAövn iv 7-15 mg/kg npspnoiwc Yia 3 npspsc

npeSviZoAövn pos 1 mg/kg/npspa

KuKAo0uo0aui6n pos 1,5-2 mg/kg/npspa

iv 15 mg/kg avä 2-3 sßöopäösc

Eni avrevSei&uv oTn xophvnon KuKAo0uo0auiônc

Me9oTpeÇdTn pos h sc h 15-25 mg/sßöopäöa

AZa9eionpivn pos h 2 mg/kg/npspa

Mycophenolate mofetil* pos 2-3 gm/npspa

©EPAnEIA ZYNTHPHZHZ

1n eniXoyh

Me9oTpeÇdTn pos h sc h 15-25 mg/sßöopäöa

AZa9eionpivn pos 2 mg/kg/npspa

2n eniXovh

Mycophenolate mofetil* pos 2-3 gm/npspa

* ХopпYnan eicraç eraanjnç évôei^nç (off-label)

EYXAPIZTIEZ:

EuxapioToujs Qspjâ rqv к. KupiaKh MnoKh, (PsujarnAôyoç, A/vrpia hojavoyAsiou NoooKojsiou AQqvaç) Kai touç kk. AnvhTpio Mnoûyna (PsujarnAôyoç, KadqyqTqç naQoAoy'iaç, laTpiKq IxoAn ЕКПА, A' nponaiôsuTiKq naQoAoyiKq KAiviKq, Attikô NoooKojsio, AGqva) Kai néTpo П. ЕФп^^ (PsujarnAôyoç, KadqyqTqç naQoAoy'iaç, laTpiKq IxoAn ЕКПА, A' nponaiôsuTiKq naQoAoyiKq KAiviKq, AaiKO rNA, AGqva) yia tiçxPQOiJsç napaTqpqosiç js та onoia ouvsßaAav orqv TsAiKq 0юу0рфшоп tqç avaoKônqoqç.

nAPAPTHMATA

nAPAPTHMA 1 Kpixnpia evepyoxnTaQ aprnpixiSaQ Takayasu Evepyoq voooq opiZeTai n ej0avian n eniSeivwan £ 2 sk twv napaKaTw:

1. ZuaTnjaTiKeQ SKSnAwaeiQ (n.x. nupeTOQ, juoaKsAeTiKeQ SKSnAwaeiQ) nou Sev jnopouv va anoSoBouv as aAAn aiTioAoYia

2. napouaia au^njevwv Ssiktwv 0AeYjovnQ (TKE)

3. napouaia anjsiwv aYYeiaKHQ 0AeYjovnQ n iaxaijiaQ (xwAoTnTa, jsiwan n anouaia a0u^ewv, KapwToSuvia, Sia0opa apTnpiaKHQ nisanQ avw n KaTw aKpwv)

4. TuniKa aYYeioYpa0iKa eupnjaTa (otsvwosiq, aveupuajaTiKn SiaTaan) KaTa Tnv aneiKovian.

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

nAPAPTHMA 2

EKTiMnon thq evepYOTnxaQ TWV ANCA aYYeiiTiSwv

H eKTipnon THQ evepYOTHTaQ thq vooou YiveTai pe th KAipaKa BVAS (Birmingham Vasculitis Activity Score,version 3).

EniAs^TE sva avriKsi|jsvo ^ovo av ano5i5srai as svspyo ayvsiiTiSa. Av 5sv unapxouv 5iarapaxsc; as Kanoia sv6rnra, sniAs^Ts «Kaveva» yia to auyKEKpipsvo opyavo-auaTr||Ja Av 6Asq oi 5iaTapaxsQ o^siAovrai as spisvouaa v6ao (svspY6Q aYYsiiTi5a nou 5sv s|j$aviaTnKs/sm5sivw9nKs tiq rsAsuraisc; 4 sp5o|a5£Q), smAsijTs «EMMENOYIA» arnv Kami 5s^ia Y^ivia

Eivai auTH n npuTn CKTiMn°n tou ao9evouq; Nai O Oxi O

Kavsva EvspY6Q v6aoQ Kavsva EvspY6Q v6aoQ

1. TcviKa O 6. KapSiaYY£iaK6 O

MuaAyisq O Anouaia a$ui;stt>v O

Ap9paAyi£Q / ap0piTi5a o BaApi5ona9sia O

nupsTOQ > 38° C o nspiKap5iri5a O

AnwAsia papouc; > 2 kg o Irn9aYxn O

2. Aep^a O MuoKap5iona9sia O

'E|j$paKTo o Iu|^opnriKn Kap5iaKn avsnapKsia O

nop^upa o 7. KoiXia O

'EAkoq o nspiroviTi5a O

rayypaiva o AipoppaYiKn 5iappoia O

AAAn 5sp|ariKr| ayy£iiTi5a o KoiAiaK6 6Ayoq iaxai|iKou runou O

3. BAevoY6voi / O$9aAMoi O 8. Ne$poi O

iTopaTiKa sAKn o Ynspraan O

rsvvnTiKa sAKn o npttirsTvoupia >1 + o

OAsypovn aisAoyovwv r| 5aKpuiKwv a5svo>v o Ai|aroupia >10 RBCs/hpf o

InpavTiKn nponmiari o Kpsarivivn opou 1,41-2,81 mg/dl* o

!KAnpiTi5a / EmaKAnpiTi5a o Kpsarivivn opou 2,82-5,64 mg/dl* o

Emns$uKiTi5a / BAs$apiTi5a / KspariTi5a o Kpsarivivn opou >5,65 mg/dl* o

06Att>an opaariQ Ai$vi5ia anwAsia 6paanQ o o Au^nan rnQ KpsarivivnQ opou >30% r| |Jsitt>an rnQ Ka9apanQ KpsarivivnQ >25% o

Payo£i5iTi5a o *Na ou^nXnpuvovTai ^ovo KaTa Tnv npuTn eKTipnon

BAapsc; a|j$ipAnaTposi5ouc; (ayy£iiTi5a/ 9p6|Pwan/£5i5pw|aTa/ai|oppayi£Q) o 9. NeupiKO ouaTnMa O Ks^aAaAYia MnviYYiTi5a o o

4. 'OTa / Piva / AapuYYaq O luYXuan o

EniaTa^n/MsiPpavsQ/EEAKn/KoKKiwiaTa o Inaapoi (6xi unspraaiKriQ airioAoYiaQ) AYYsiaK6 sYKs^aAiK6 snsia65io o o

napappivoKoAniTi5a o MusAona9sia o

YnoyA^TTi5iKn aTsvwan o napsan KpaviaKou vsupou o

BapnKoia aywyi|6TnTaQ o Aia9nriKn nspi^spiKn vsupona9sia o

Nsupoaia9nrr|pioQ papnKoTa o noAAanAn |ovovsupiri5a o

5. OupaKaQ O

Iupiy|6Q o 10. AXXa O

'OZoi n KoiA6TnT£Q o a. o

nAsupiTiKn auAAoyn / nAsupo5uvia o p. o

Ain9n|aTa o o

Ev5oPpoyxiKn pAapn o 5. o

MaZiKn ai|6nTuan/Ku^sAi5iKn aipopayia AvanvsuariKri avsnapKsia o o MONO EMMENOYZA NOIOI: (EniAs^rs av 6Asq oi 5iarapaxsQ ano5i5ovrai as sppsvouaa v6ao) □

rAQZZAPIO KAI BAGMOAOrHZH BVAS

OSnyieQ Yia Tn oupnAnpwon tou BVAS

1. Oi SKSnAwaeiQ TnQ voaou PaBjoAoYouvTai povo av anoSiSovTai oe evepYO aYYeiiTiSa. H sKSnAwan TnQ voaou Sev npensi va PaBjoAoYeiTai av unapxsi Aoyikh evaAAaKTiKn aiTia Yia Ta aujnTwjaTa, n.x. Aoijw^n, 0apjaKsuTiKn avTiSpaan, aAAn auvoanpoTnTa.

2. EniAe^Ts «Ejjevouaa voaoQ» eav oAsq oi SiaTapaxeQ anoSiSovTai as as evepYo (aAAa oxi vsosj0aviZojevn n sniSeivoujevn) aYYSiiTiSa.

3. ria Tn aujnAnpwan opiajevwv ano Ta nsSia, sivai anapaiTnTn n SKTijnan ano iaTpouQ aAAwv siSiKoTnTwv n Ta anoTsAeajaTa epYaaTnpiaKwv n ansiKoviaTiKwv s^STaaewv. Me e^aipean auTeQ tiq nepinTwasiQ, n 0opja npensi va aujnAnpwvsTai oto auvoAo TnQ Tn otiyjh TnQ SKTijnanQ.

4. Ta nsSia TnQ KpsaTivivnQ opou aujnAnpwvovTai jovo KaTa Tnv npwTn eniaKS^n.

5. AvTiKsijsva nou anjeiwvovTai je aaTspiaKo (*) Sev sivai aujPaTa je «ejjevouaa» voao. AuTeQ oi SKSnAwaeiQ auviaTouv navToTs vsosj0aviZojevn n sniSevoujevn voao oTav anoSiSovTai as evepYo aYYSiiTiSa.

EKSnAwon OpiopOQ Eppevouoa Nea / EniSeivoupevn

1. reviKa MeyioTeq Tipeq 2 3

MUOAYISQ AAyoq otouq pUEQ 1 1

ApGpaAYisQ n ap9piTi5a AAyoq n ^AsYpovn twv ap9pwosa>v 1 1

nupsTOQ > 38°C TsKpnpiwpsvn 9sppopsTpnon oTopaToQ n paoxaAnQ. Av psTpn9si 9sppoKpaoia op9oi>, to opio sivai 38.5°C 2 2

AnwAsia papouQ > 2 kg AnwAsia ^npou owpaTiKou papouQ x^P'Q 5iaiTnTiKo nspiopiopo 2 2

2. Acppa MeyioTeq Tipeq 3 6

'Ep^paKTo nspioxn iotikhq vsKpwonQ n unovi>xisQ aipoppaYisQ 1 2

npo^upa Yno5sppaTiK£Q n unopAsvoYovisQ aipoppaYisQ anouoia TpaupaTOQ 1 2

'EAkoq Auon oTn ouvsxsia tou 5sppaTOQ 1 4

raYYpaiva EKTSTapsvn IOTIKH vsKpoion 2 6

AAAn 5sppaTiKr| aYYsiiTi5a AiKTu^Tn nsAiwon, uno5opia oZi5ia, oZW5sq spu9npa KAn 1 2

3. BXevoYovoi / 0$9aApoi MeYioTeq Tipeq 3 6

iTopaTiKa sAKn / KoKKiwpaTa A$9w5nQ oTopaTiTi5a, pa9sia sAKn, unspnAaoia ouAwv («5iKnv $paouAaQ») 1 2

rsvvnTiKa sAKn 'EAKn YsvvnTiKwv opYavwv n nspivsou 1 1

OAsYpovn oisAoYovwv n 5aKpuiKwv a5svtt>v OAsYpovn oisAoYovmv h 5aKpuiKwv a5svtt>v 2 4

InpavriKn nponT^on nponmion o$9aApoi> > 2 mm 2 4

!KAnpiTi5a / EnioKAnpiTi5a OAsYpovn tou oKAnpou xiTwva 1 2

Emns$uKiTi5a/BAs$apiTi5a/ KspaTiTi5a OAsYpovn tou srans^uKOTa, pAs^apwv n KspaTosi5ouQ - pn o^siAopsvn os ^npo^9aApia 1 1

©oAoion opaonQ Ensi5sivtt>on TnQ onTiKHQ o^uTnTaq 2 3

Ai$vi5ia anwAsia opaonQ* O^sia anwAsia TnQ opaonQ * 6

PaYosi5iTi5a OAsYpovn tou paYosi5ouQ xiTwva (ipi5a, aKTiv^To owpa, xopsiosi5sQ) 2 6

BAapsQ ap^ipAnoTposi5ouQ (aYY£iiTi5a / 9popP^on / si;i5pWpaTa / aipoppaYisQ) EiKova aYYsiiTi5aQ ap^ipAnoTposi5ouQ oTn kAivikh s^ETaon ("sheathing" of retinal vessels) h oTn ^AouopoaYYsioYpa^ia, 9popp^on apTnpiwv n ^Aspwv ap^ipAnoTposi5ouQ, paAaKa s^i5pwpaTa/ ap^ipAnoTposi5iKn aipoppaYia 2 6

of RHEUMATOLOGY 2

E Л Л H N I K H PEYMATOЛOГIA 2015

EröqAuon OpiopóQ Epncvouoa Nca / Eпlбelvoúиevn

4. 'Ото / Píva / Лápuvvaç Mévloтeç ^^ÉQ З б

Emora^ / Msußpávsc / 'ЕЛ^ / to^i^uara Aiua^psc, ßЛsvопuшбslc piviKsc sKKpÍosic, кaфsоslбsÍc usußpávsc пои ouxvá aпофpáooоuv тlc piviKsc GaЛáusc, piviKá еЛкп п коккlшuaтшбslc ßЛáßsc пои avaбslкvúоvтal oтn plvоoкóпnon 2 4

ПapapplvокоЛп¡тlбa EuaioGnoÍa п áЛYоc oтnv пsplоxп tov пapapplvíшv гоЛпом (ouxvá smßsßaitt>usvn us a^irâvion) 1 2

EröqAuon OpiopóQ Ennévouoa Nca / Eпlбelvoúиevn

YпоYЛшттlбlкr| oт£vшon lupiYuóc п ßpáYXоC офslЛóusva os фЛsYUоvr| Kai oт£vшon тnc uпоYЛшттlбlкr|C пsplоxпc oтnv ЛapuYYоoкóпnon 3 б

Bapnroïa aYo^uó^TOc AпшЛsla a^Hc офslЛóusvn os ouuusтоxп тои uSoоu OITOC (smßsßaiwusvn us aкооusтpÍa) 1 3

NsupоaloGnтпplоc ßapnK6ia AпшЛsla a^Hc офslЛóusvn os ßЛáßn тои aкоuoтlкоú vsúpоu п ^хЛю^ ßЛáßn (smßsßaiwusvn us aкооusтpÍa) 2 б

5. Ouparaç Mévloтeç ^^ÉQ З б

lupiYuóc lupiYuóc oтnv ^iviKÍ sÇsraon 1 2

'ОСо п коlЛóтnтsc Nssc ßЛáßsc oтоv aпslкоvloтlкó SЛsYXо * 3

ПЛsuplTlкr| ouЛЛоYП / пЛsupобuvía ПЛsuplTlкó áЛYоc Kai/п í^c тplßпc oтnv i^ivi^ sÇsraon, aктlvоЛоYlкá sraßsßaiwusvn пЛsuplTlкr| ouЛЛоYП 2 4

Д^п^та 'Опо^ бюпют^о^га os aктlvоYpaфÍa п a^vi^ тоuоYpaфÍa GwpaKa 2 4

EvбоßpоYXlкп ßЛáßn EvбоßpоYXlкóc фsuбоÓYKоc п sЛкштlкSc ßЛáßsc. Iqjeíwoq: OuaЛSc oтsvштlкSc ßЛáßsc кaтaYpáфоvтal oто бsíктn xpоvlóтnтac VDI. Oi uпоYЛшттlбlкSc ßЛáßsc va кaтaYpáфоvтal oтnv svóтnтa 4. 2 4

MaZiKr aiuórnuon / кuфsЛlбlкr| aluоppaYÍa Msííoiv пvsuuоvlкп aluоppaYÍa, us usтaкlvоúusva пvsuuоvlкá бlnGпuaтa 4 б

Avarosuo™! avsnápKsia AváYKn unxavlкоú asplouоú 4 б

6. Kapбlavvelaкó McYloтeç З б

AпоuoÍa oфúÇsшv KЛlVlкá бlaпloтшuSvn aпоuoÍa TOp^spiKtiv ap^piaK^v oфúÇsшv os опоlобr|Потs áкpо 1 4

BaЛßlбопáGsla ^ivi^ п uпspnxоYpaфlкп бип^а^ ßЛáßnc oтnv aоpтlкп / ulтpоslбп / пvsuuоvlкr| ßaЛßÍбa 2 4

Пsplкapбíтlбa Пsplкapбlaкó áЛYоc / i^c тplßпc oтnv kAIV^! sÇsraon 1 3

IтnGáYxn TumKó loтоplкó GaipaKirou áЛYоuc пои обnYsÍ os SuфpaYua тои uuокapбÍоu п oтnGáYxn 2 4

MuокapбlопáGsla InuavriKá sпnpsaouSvn кapбlaкr| ЛslтоupYÍa офslЛóusvn os uпокlvnoÍa тои TOixwuaTOc tov ro^itiv, sraßsßaiwusvn us uпspnxокapбlOYpaфÍa 3 б

Iuuфоpnтlкп кapбlaкп avsnápKsia Kapбlaкп avsпápкsla ßáosl ioTOpiroù п ^ivi^c sÇsraonc 3 б

7. KoiAía Mévloтeç тфс^ 4 9

ПsplтоvÍтlбa TumKó коlЛlaкó áЛYOc svбslктlкó ouuusтоxпc тои пsplтоvaÍоu 3 9

A^ppaYiKÍ бláppоla Пpóoфaтnc svapínc 3 9

KоlЛlaкó áЛYоc loxalUlкоú типои TumKó rabiará áЛYOc svбslктlкó ioxaiuíac TOU svтSpоu, smßsßaiwusvnc us aпslкóvlon п бlSYXSlpnтlкá 2 б

EKSnXuon OpiO^OQ E^MCvouoa Nca / EniSeivou^evn

8. Ne$poi McYiOTeQ Ti^EQ 6 12

Ynspraan AiaaroAiKH >95 mm Hg 1 4

npwTsTvoupia >1+ arn ysvikh sJjsraan oupwv H >0.2g/24 hours 2 4

Aiparoupia «Msrpia» arn ysvikH oupwv H >10 RBCs/phf, auxva auvo5su6|svn |s spu9poKurrapiKouc; KuAiv5pouQ 3 6

Kpsarivivn opou 1,41-2,81 mg/dl M6vo arnv npwrn sKTipnan 2 4

Kpsarivivn opou 2,82-5,64 mg/dl 3 6

Kpsarivivn opou a 5,65 mg/dl 4 8

>30% au^nan arnv Kpsarivivn H >25% sAarrwan arnv Ka9apan KpsarivivnQ* npoo5suTiKH sni5sivwan rnQ vs$piKr|Q AsiroupYiaQ. Mnopsi va xpnai|-ionoin9si as Ka9s sKTipnan, sav n vs$piKr| AsiroupYia sxsi sni5sivw9si as axsan |is nponYoupsvn ri|H * 6

EKSnXuon OpiO^OQ E^MCvouoa Nca / EniSeivou^evn

9. NeupiKO ouoTnMa MEYiOTeQ Ti^CQ 6 9

Ks^aAaAYia AauvH9iarn Kai sppsvouaa Ks^aAaAYia 1 1

MnviYYiri5a KAIVIKSQ sv5si^siQ inviYYiaiou 1 3

luYXuan AiarapaxsQ npoaavaroAiapou, |vHinQ H aAAwv vonriKwv AsiroupYiwv anouaia israpoAiKwv, ^uxiarpiKwv ^ap|aKsuriKwv H to^ikwv airiwv 1 3

Inaapoi (6xi unspraaiKHQ airioAoYiaQ) KAIVIKSQ H nAsKTposYKs$aAoYpa$iKsQ sv5si^siQ na9oAoYiKHQ nAsKTpiKHQ 5paarnpi6TnTaQ rou sYKs^aAou 3 9

AYYsiaK6 syks$oAik6 snsia65io EariaKa vsupoAoYiKa aniisia 5iapKsiaQ >24 wpwv nou ano5i5ovrai as aYYsiaK6 snsia65io rou KNI 3 9

MusAona9sia KAiviksq H ansiKoviariKsQ sv5si^siQ auiisroxHQ rou vwriaiou pusAou 3 9

napsan KpaviaKou vsupou KAiviksq sv5si^siQ napsanQ KpaviaKou vsupou -(pa9|oAoYsiars rnv napsan rnQ VII sYKs$aAiKr|Q auZuYiaQ wq vsupoaia9nrr|pio papnKoTa, in pa9|oAoYsirs napsasiQ o$9aA|joKivnTiKwv vsupwv sav o^siAovrai as nisariKa $aiv6|jsva) 3 6

Aia9nTiKH nspi^spiKn vsupona9sia YnoKsi|sviK6 aia9nriK6 sAAsi||a xwpiQ 5sp|oTo|iaKH KaravopH 3 6

noAAanAH |ovovsupiri5a MovHpnQ H noAAanAH napsan KivnriKwv vsupwv 3 9

Ta 5ia0opa OToixeia nou nepiAapPavovrai oto epYaAeio evepYOTnTaQ BVAS Ta^ivopouvTai oe peiZova Kai eAaooo-va. Me Paon auTa, npoKumouv oi aKoAou9oi opiopoi.

Opiopoi 'YQsoq

KaAwQ eAeYXopevn voooq

- Yno aY^YH O0eon: HpepHoia Soon npeSviZoAovnQ < 10 mg Kai BVAS <1 Yia SiaoTnpa > 6 pqveQ

- Y0eon eAeu9epn aYWYHQ: Kapia aY^YH Yia > 6 pqveQ

of RHEUMATOLOGY

EAAHNIKH PEYMATOAOriA

YnoTponq

Noooq, n onoia eAsYxoTav KaAwQ (je n xwpiQ aYwYn) Kai nAeov eivai evepYH

- MeiZwv unoTponn: Au^nan as > 1 jeiZova aToixeia tou BVAS

- EAaaawv unoTponn: Au^nan as > 1 eAaaaova aToixeia tou BVAS, anouaia jsiZovoQ aToixsiou AvOeKTiKq voaoq:

E^sAiaaojevn voaoQ nou Sev avTanoKpivsTai aTnv Bepaneia Kai Sev exei sniTsuxBsi u0ean neptopta^evq voaoq:

- Anouaia evepYou aneipajaTovs0piTiSaQ (0uoioAoYiKn KpeaTivivn opou, anouaia spuBpoKuTTapiKwv KuAivSpwv H npwTs'ivoupiaQ) Kai

- Anouaia jsiZovoQ npoaPoAnQ aneiAnTiKHQ Yia Zwtiko opYavo n Tn Zwn twv aaBevwv (n.x. nveujoviKn aijoppaYia, aYYSiiTiSa KNZ, sniSeivoujevn aoPapn nspi0spiKn vsuponaBsia, o0BaAjiKoQ ^suSooykoq, aijoppaYia YaaTpe-vtspikou, nepiKapSiTiSa, juoKapSiTiSa).

Zopapq/yeviKcuyevn voaoq:

OAoi oi unoAoinoi aaBsveiQ, nAnv auTwv nou nepiYpa0ovTai aTnv nspiopiajevn voao.

nAPAPTHMA 3

TPOnOnOIHZH THE AOZHZ THE KYKAOOfiZOAMIAHZ ANAAOrfiZ OAOY XOPHrHZHZ, HAIKIAZ, NEOPIKHZ AEITOYPriAZ KAI APIOMOY WBC

KuKAo0wo0aMi6n ano tou ot6mqtoc (pos)

• Meyioth Soon: 200 mg npepnoiwQ

• HAiKia > 60 eTwv: peiwon SoonQ KaTa 25%

• HAiKia > 75 eTwv: peiwon SoonQ KaTa 50%

• 'EAeYXOQ pe YeviK| aipaTOQ Ka9e ePSopaSa tov npwTo p|va, ava Suo epSopaSeQ to SeuTepo Kai TpiTo pi|va Kai aKoAou9wQ ava p|va. EAaTTwon TnQ SoonQ, av o api9poQ twv WBC<4.000/mm3.

Ev6o0A¿P^a KUKAo$wo$aMiSn

Tpononoinon Soonc ev5o$A¿Plac KUKAo$wo$aMiSnc Paoet nAiKiac Kai ve$piKnc AetToupyiac (npwTQKoAo CYCLOPS)

HAiKia (¿Tn) KpeaTivivn opoU (mg/dL)

1.7 - 3.4 3.4 - 5.6

<60 15 mg/kg/wan 12.5 mg/kg/wan

60 - 70 12.5 mg/kg/wan 10 mg/kg/wan

>70 10 mg/kg/wan 7.5 mg/kg/wan

Tpononoinon Soonc Paoet nAiKiac Kai veQptKqc Aeiroupyiac (unoAoytoMoc Paoei emfoaveiac o^paToc)

AoooAoYia: 0.75 g/m2 ava 3-4 epSopaSeQ. EAaTTwon TnQ apxiK|Q SoonQ oe 0.5 g/m2 oe:

- HAiKia > 60 eTwv |

- GFR < 20 ml/min/1.73 m2

Tpononoinon Soonc tioewv KUKAo$wo$aMiSnc Paoet apiSpou AeuKtiv atMooQatpiwv

• MeTpnon api9pou WBC Tnv qjspa tqq npoypajjaTiojevqq woqq q jia qjspa npiv. Eav WBC < 4 x 109/L, TOTe:

- AvaoToA| TnQ SoonQ ewQ otou WBC>4 x 109/L, Kai

- EAaTTwon TnQ SoonQ TnQ enopevnQ wonQ KaTa 25%

• MeTpnon WBC jsm^u 10nQ Kai 14n qjepaq jsto tqv woq

Eav, to vaSip twv WBC<3 x 109/L (aKopn Kai av WBC aKpiPwQ npiv Tnv won >4 x 109/L), ToTe Yia vaSip WBC:

- 1-2 x 109/L: eAaTTwon TnQ SoonQ TnQ enopevnQ wonQ KaTa 40% oe oxeon pe Tnv nponYoupevn

- 2-3 x 109/L, eAaTTwon TnQ SoonQ TnQ enopevnQ wonQ KaTa 20% oe oxeon pe Tnv nponYoupevn

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

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