Evidence-based treatment for systemic sclerosis Текст научной статьи по специальности «Клиническая медицина»

of RHEUMATOLOGY

EAAHNIKH PEYMATQAOriA

26 1

2015

ANAXKOnHXH REViEW

Gepaneia luornpariKou iKXnpoSepparoQ Baoiopevn oe EvSei^eiq

BaoiXiKr]-KaXXi6nr| Mnoupvia MD, neipoq n. I0nKaKr|Q, MD, PhD

MovaSa PeupciToXoYiaq A' nponcu6euTiKr]Q naBoXoyiKHQ KXiviKHQ, Aci'iko NoooKopelo, laipiKr] IxoXfi, EGviko Kai KanoSioTpiaKo navenioirpo ABnvwv

nEPIAHYH

H Gepaneia tou ouornpariKou oKXnpoSepparoQ napouoiaZei onpavriKeQ npoKXqoeiQ Kai iSiaiTepornreQ. EXXei^ei 0appaKwv nou rpononoiouv ouvoXiKa Tn 0uoikH nopeia rnQ vooou, n GepaneuTiKH npooeYYion npooavaroXiZerai ornv avTiperwnion rwv oupnrwparwv ano Ta opyava nou ¿xouv npoo|3XnGei Kara nepinrwon. £rnv napouoa epYaoia npoonaGqoape va ouornparonoinooupe Tn |3aoei evSei^ewv GepaneuriKH avriperwnion rwv Kupiorepwv eKSnXwoewv tou ouornpariKou oKXnpoSepparoQ, a^ionoiuvraQ tiq KareuGuvrHpieQ oSnYieQ rnQ EULAR-EUSTAR Kai Ta SiaGeoipa |3i|3XioYpa0iKa SeSopeva. ZuYKeKpipeva, oTnv npoo|3oXn tou SepparoQ, oe opoYoviTiSa H apGpiriSa pnopei va xopnYnGei peGorpeEiarn oe ouvSuaopo pe piKpH Soon KopTiKoeiSwv. ria Tnv avriperwnion tou 0aivopevou Raynaud Kai rwv SaKruXiKwv eXKwv xopnYouvTai anoKXeioreQ SiauXwv aopeoriou oe u^nXeQ SooeiQ Kai oe avGeKTiKeQ nepinrwoeiQ evSo0Xe|3ia avaXoYa npooraKuKXivnQ. £rnv npoXn^n rwv SaKruXiKwv eXKwv exei Geon Kai to bosentan. H nveupoviKH aprnpiaKH unepraon avTiperwniZerai avaXoYa pe to XeiroupYiKo oraSio tou aoGevouQ pe avraYwvioreQ unoSoxewv evSoGnXivnQ-1, avaoroXeiQ 0wo0oSieorepaonQ-5, avaXoYa npooraKuKXivnQ H ouvSuaopouQ aurwv. ria Tn Siapeon nveupoviKH ivwon npoTeiveTai n xopHYnon KuKXo0wo0apiSnQ iv H per os pe H xwpiQ ano tou oroparoQ KopriKoeiSH Yia 6-12 pHveQ.

Qq Gepaneia ouvrHpnonQ xpnoiponoieiTai n aZaGeionpivn H to puKo0aivoXiKo o^u. Irn ve0piKH Kpion tou oKXnpoSepparoQ SiSovrai avaoroXeiQ tou perarpenriKou evZupou rnQ aYYeioTaoivnQ. Oi eKSnXwoeiQ ano to YaorpevrepiKo avriperwniZovTai pe avaoroXeiQ avrXiaQ npwroviwv, SopnepiSovn, kukXiko oxHpara xopHYnonQ avri|3ioTiKwv H Kai oKrpeoriSn. H reXiKH eniXoYH rou KaraXXnXorepou Yia rov KaGe aoGevH GepaneuriKou oxHparoQ paoiZerai ornv kXivikh Kpion rou GepanovroQ.

Yneu8uvoQ aAAriAoypa^iaQ:

Ka8nvnTnQ nexpoQ n. I$r|KaKr|q

MovaSa PeupaToAoyiaQ A' nponaiSeuTiKiiq

na8oAoyiKHQ KAiviKiQ,

AaiKo NoaoKopeio, laTpiK| 2xoAi

E8viKo Kai KanoSiorpiaKo naveniarnMio A8r|vwv

Tr|A:213-2061061, 213-2061307

Oa£: 210-7791839

E-mail: psfikakis@med.uoa.gr

Corresponding author:

Professor Petros P. Sfikakis

Rheumatology Unit, 1st Departemnt of Propedeytic

Internal Medicine

Laiko Hospital, Medical School

National kapodistrian University of Athens, Athens

Tel: 213-2061061, 213-2061307

Fax: 210-7791839

E-mail: psfikakis@med.uoa.gr

Mediterr J Rheumatol 2015; 26(1): 56-63

Ae^eiQ-KXeiSia: Gepaneia, SaKTuXiKa eXKn, peGorpe^arn, veQpiKq Kpion oKXnpoSeppiaQ, ouornpariKH oKXHpuvon

Evidence-based treatment for systemic sclerosis

Vasiliki-Kalliopi Bournia, MD, Petros P. Sfikakis, MD, PhD

Rheumatology Unit, 1st Departemnt of Propedeytic Internal Medicine, Laiko Hospital, Medical School, National Kapodistrian University of Athens, Athens

ABSTRACT

The treatment of systemic sclerosis is fraught with many challenges. The therapeutic approach targets affected organs, since there is no medications that modify the disease itself. In this review we tried to present the therapeutic management of main manifestations of systemic sclerosis based on the EULAR-EUSTAR recommendations and published data. In particular, in skin involvement, serositis, or arthritis, methotrexate along with small dose of corticosteroids can be used. For Raynaud's phenomenon and digital ulcers, high doses of calcium channel blockers, and inrefractory cases, IV prostacyclin analogues are used. For the prevention of digital ulcers, bosentan can be used. Pulmonary arterial hypertension is managed according to functional stage with inhibitors of endothelin receptors, inhibitors of phosphodiesterase-5, prostacyclin analogues, or their combination. For interstitial lung disease, cyclophosphamide IV or per os with or without corticosteroids are used for 6-12 months. As maintenance treatment azathioprine or mycophenolate is used. For sceroderma renal crisis, inhibitors of angiotensin converting enzyme are used. For gastrointestinal manifestations, proton pump inhibitors, domperidone, cyclic administration of antibiotics or octreotide are used. The therapeutic choice for a particular patient is based on clinical judgement.

Mediterr J Rheumatol 2015; 26(1): 56-63

Keywords: Systemic sclerosis, treatment, methotrexate, bosentan, digital ulcer, scleroderma renal crisis

of RHEUMATOLOGY 1

E Л Л H N I К H РЕУМАТОЛОПД 2015

Eioayuyn

To ouoTnjaTiKö oKAnpöSepja eivai jia xpövia no-Лиоиотпмат1кп vöooq пои xapaKirpZeTai anö ivw-on тои 6ep|jaTOQ Kai twv eowTepiKwv opYavwv. To vöoriHa афора KupiwQ YuvaiKeQ jeoriQ nAiKiaQ, n 5e enimwon тои OTnv EAAaSa npooeYYiZei та 11 vea ne-рютат1ка/106 nAn9uojoü кат' ¿toq, evw о eninoAa-ojöQ eivai 154 nepinTwoeiQ/106 nAn9uojoü.1 H 9epaneia tou ouoTnjaTiKoü OKAnpoSepjaToQ na-pouoiaZei onjavTiKeQ npoKAnoeiQ Kai iSiaiTepöTnTeQ. Ka9wQ, jexpi OTiYjnQ, n na6oYeveia tou voonjaToQ napajevei aYvwoTn, 5ev ¿xei ßpe9ei Kanoio фар-jaKo nou va Tpononoiei ouvoAiKa тп фuоlкП nopeia TnQ vöoou. AvTi9eTa, n öepaneuTiKn npooeYYion eivai npooavaToAiojevn отп OToxeujevn avTijeTwnion twv oujnTwjaTwv tou ao9evoüQ, je ßaon Ta öpYa-va nou ¿xouv npooßAnöei KaTa nepimwon. nepav TnQ noAuouoTnjaTiKnQ фüonQ tou voonjaToQ Kai TnQ eTepoYeveiaQ отп ßapüTnTa TnQ npooßoAnQ, тп АПФп 9epaneuTiKüv kAivikwv aпoфäoewv Suoxepaivei n on-javTiKn To^iKöTnTa noAAwv anö tiq eфapJoZöJeveQ 6epaneieQ, oe ouvSuaojö je to opiaKö öфeAoQ nou evioTe auTeQ napexouv, önwQ evSeiKTiKa avaф¿peтal Yia Tnv nepimwon TnQ кuкAoфwoфaJÍ6nQ отп 9epa-neia TnQ nveujoviKnQ ivwonQ tou OKAnpoSepjaToQ2. TeAoQ, n iöia n a^ioAöYnon twv anoTeAeojaTwv TnQ 6epaneiaQ jnopei va KaTaoTei npoßAnjaTirai aфoü, xapaKTnpioTiKa, n SepjaTiKn npooßoAn, nou TuniKa a^ioAoYeiTai wq jeTpo ßapüTnTaQ oe ao6eveiQ je ouoTnjaTiKö oKAnpööepja, ¿xei Tnv таоп va ßeATiwve-Tai aKöjn Kai xwpiQ aYwYn jeTa anö jia apxiKn фаоп ¿vTovnQ eniSeivwonQ3.

AKöjn Kai je auToüQ touq nepiopiojoüQ, n npöoSoQ nou ¿xei onjeiw9ei Ta TeAeuTaia ¿тп отп SiaYvwon Kai 9epaneia tou ouoTnjaTiKoü oKAnpoSepjaToQ avTa-vaKAaTai отп ßeATiwon TnQ enißiwonQ twv ao9evwv, oe oxeon je to napeA9öv4. MaAioTa, avaф¿peтal öti n Ka9iepwon TnQ XPnonQ avaoтoA¿wv tou jeTaTpenTi-koü evZüjou TnQ aYYeioTevoivnQ jeTaTönioe to Küpio ßapoQ TnQ oxeTiZöjevnQ je to ouoTnjaTiKö oKAnpö-Sepja voonpöTnTaQ Kai 9vnTöTnTaQ anö тп veфplкп Kpion tou oкAnpo6¿pJaтoQ OTnv nveujoviKn ivwon Kai Tnv nveujoviKn apTnpiaKn uräpTaon, nou пA¿ov anoTeAoüv tiq KüpieQ aiTieQ 9avaTou anö to vöonja5. To 2009 5njooieü9nKav Yia npwTn фopä KaTeu9uvTn-pieQ oSnYieQ Yia тп 9epaneia tou ouoTnjaTiKoü окАп-po6¿pJaтoQ anö Tnv OjaSa Yia tiq KAiviräQ AoкlJ¿Q Kai Tnv Epeuva oto iKAnpööepja TnQ EupwnairaiQ eTaipeiaQ peujaToAoYiaQ (EULAR-EUSTAR) 67. Zto napov ap9po xpnoijonoin9nKav wq ßaon oi oSnYieQ TnQ EULAR-EUSTAR, evw avaoKonn9nKav oi npoTa-oeiQ aAAwv ojaSwv eiöiKüv, Ka9wQ Kai eпlAeYJ¿veQ SnjooieüoeiQ anö тп 6la9¿olJn ßlßAlOYpaфía nou aфopoüv тп 9epaneia tou ouoTnjaTiKoü oкAnpo6¿p-jaToQ. ZuYKeKp^va, oi oSnYieQ nou nepiAajßävovTai

napaKÖTw aфopoüv тп 9epaneia twv aKöAou9wv eK-SnAwoewv tou ouoTnjaTiKoü oкAnpo6¿pJaтoQ: a. tou фalvoJ¿vou Raynaud Kai twv SaKTuAiKwv eAKwv, ß. TnQ nveujoviKnQ apTnpiaraiQ uп¿pтaonQ, Y. TnQ 5i-ajeonQ nveujoviKnQ ivwonQ, 5. TnQ veфplкпQ KpionQ, e. TnQ npooßoAnQ tou YaoTpevTepiKoü ouoTnjaToQ, от. TnQ npooßoAnQ tou 6¿pJaтoQ, Z. TnQ npooßoAnQ twv ap9pwoewv Kai twv opoYövwv uJ¿vwv Kai n. TnQ npooßoAnQ TnQ KapSiaQ.

A. Oepaneia Фагоон^ои Raynaud Kai AaKxuAiKÜv EAKWV

• Oi AпoкAeloт¿Q SiaüAwv aoßeoTiou tou Tünou TnQ SiuSponupiSivnQ 9ewpoüvTai 9epaneia npwTnQ YpajjnQ Yia тп jeiwon TnQ ¿vTaonQ Kai TnQ ou-xvöTnTaQ tou фalvoJ¿vou Raynaud oe ao9eveiQ je ouoTnjaTiKö oKAnpöSepja Kai jnopoüv va xopn-Yn9oüv oe SöoeiQ nou SuvaTöv va ф9ävouv Tnv J¿-YiOTn aveKTn (nx. vlфe6lпívn 120 mg njepnoiwQ) 8.

• Ta ev6oфA¿ßla avaAoYa npooTaKuKAivnQ (nx. iAo-npöoTn oe 5öon 0.5-2 ng/kg/min Yia 3-5 ouvexö-jeveQ nJ¿peQ) jnopoüv va xpnoijonoin9oüv wq aYwYn SeüTepnQ YpajjnQ Yia тп 9epaneia TnQ ßa-piaQ JopфПQ фalvoJ¿vou Raynaud oe ao9eveiQ je ouoTnjaTiKö oKAnpöSepja, nou 5ev avTanoKpivo-vTai отп AпФn anoKAeioTüv SiaüAwv aoßeoTiou9.

• Oi aпoкAeloт¿Q SiaüAwv aoßeoTiou oe SöoeiQ nou SuvaTöv va ф9ävouv Tnv J¿Yloтn aveKTn (nx. vlфe-Sinivn 120 mg njepnoiwQ), jnopoüv va xopnYn-9oüv Yia тп enoüAwon twv SaKTuAiKüv eAKÜv.

• Ta ev6oфA¿ßla avaAoYa npooTaKuKAivnQ (nx. iAo-npöoTn oe 5öon 0.5-2 ng/kg/min Yia 3-5 ouvexö-jeveQ nJ¿peQ) jeiwvouv tov api9jö twv SaKTuAi-küv eAKüv Kai 9ewpoüvTai e^ioou anoTeAeojaTiKa je touq aпoкAeloт¿Q SiaüAwv aoßeoTiou Yia Tnv npoaYwYn TnQ enoüAwonQ twv eAKÜv910.

• To bosentan (jn eKAeKTiKöQ avTaYwvioTnQ twv uпo6ox¿wv ev5o9nAivnQ-1) 5ev ¿xei pöAo отп 9e-paneia twv n5n unapxövTwv evepYwv eAKÜv aAAa jövo OTnv пpoфüAa^n KaTa TnQ SnjioupYia v¿wv eAKüv1112. Mnopei va xpnoijonoin9ei oe ao9eveiQ önou n 9epaneia je aпoкAeloт¿Q SiaüAwv aoßeoTiou n npooTavoeiSn 5ev nTav anoTeAeojaTiKn.

• ria touq avaoToAnQ фwoфo6leoтepäonQ-5 (nx. sildenafil) 5ev unapxouv 6lпA¿Q-тuфA¿Q n eAeYxö-jeveQ je eiKoviKö фäpJакo JeA¿тeQ отп 9epaneia twv eAKüv Kai Yia auTö 5ev ouvioTaTai n xpnon touq oTa ¿AKn twv SaKTüAwv. E^aipeon anoTeAei to tadalafil, to onoio oTa nAaioia jiaQ jiKpnQ Tuxai-oпolnJ¿vnQ JeA¿тnQ, ¿Sei^e ßeAтlWJ¿vn enoüAwon Kai пpöAnФn TnQ eJфävlonQ v¿wv SaKTuAiKüv eA-kwv oe ouYxopnYnon je v^eöinivn n aAAa aYYeio-SiaoTaATiKa13.

B. nveupoviKH ApxnpiaKn Ynepxaon

H ôiâYvwori TnQ nveupoviKHQ apTnpiaKHQ unépTaonQ TÎ9eTai unoxpewTiKâ pe ôeÇiô KapôiaKô Ka9eTnpia-opô, e0ôoov ôianioTw9oûv Ta napaKâTw eupHpaTa: a) péon nieon nveupoviKHQ>25 mmHg, ß) nieon evo0H-vwonQ nveupoviKüv Tpixoeiôwv<15 mmHg, y) avTi-oTaon oTnv nveupoviKH apTnpia>3 povâôeQ Wood, pe

0uoioÀoYiKn H eÀaTTwpévn KapôiaKH napoxH14.

Ztov nivaKa 1. 0aiveTai n Ta^ivôpnon twv ao9evwv pe nveupoviKH unépTaon ßaoei tou ÀeiToupYiKoû oTaôiou KaTâ New York Heart Association (NYHA). ZTnv napoûoa 0äon unâpxouv ßißAioYpa0iKä ôe-ôopéva nou TeKpnpiwvouv Tnv anoTeÀeopaTiKôTnTa tou bosentan1516, tou sildenafil17,18, tou unoôopiou treprostinil19 Kai Tnç ouvexoûQ ev5o0AeßiaQ xopHYn-onQ enonpooTevôÀnQ20 oTn 9epaneia TnQ oxeTiZô-pevnç pe to ouoTnpaTiKô oKÀnpôôeppa nveupoviKHQ apTnpiaKHQ unépTaonQ.

Aeôopévou ôti otiq nepiooôTepeQ peÀéTeQ nou oôH-Ynoav oTnv éYKpion 0appâKwv Yia Tn 9epaneia TnQ nveupoviKHQ apTnpiaKHQ unépTaonQ oupnepieÀH09n-oav Tôoo ao9eveÎQ pe iôiona9H vôoo, ôoo Kai ao9e-veÎQ pe nveupoviKH apTnpiaKH unépTaon oxeTiZôpevn pe oKÀnpôôeppa H âÀÀa vooHpaTa tou ouvôeTiKoû iotoû, npoTeiveTai n enéKTaon tou 9epaneûTiKoû aÀ-Yopi9pou Yia Tnv iôiona9H nveupoviKH unépTaon Kai oTnv nepinTwon TnQ oxeTiZôpevnQ pe oKÀnpôôeppa nveupoviKHQ apTnpiaKHQ unépTaonQ. Qq eK toûtou npoTeivovrai Ta aKôÀou9a21 :

• H ßaoiKH unooTnpiKTiKH aYWYH nou KaTâ nepi-nTwon pnopei va xopnYn9ei otouq ao9eveÎQ pe nveupoviKH apTnpiaKH unépTaon oe éôa0oQ ou-oTnpaTiKoù oKÀnpoôéppaToQ, пeplÀapßâvel: a. xo-pHYnon ôioupnTiKûv e0ôoov unâpxouv eupHpaTa oupßaTä pe ôe^iâ KapôiaKH avenâpKeia/KaTaKpâ-Tnon uypwv, ß. o^uYovo9epaneia oe ao9eveÎQ pe pepiKH Tâon 02 oto apTnpiaKô aipa <60mmHg Kai Y. ôiYoÇivn Yia Tnv eпlßpâôuvon KoÀniKHQ Taxuap-pu9piaQ.

• Ze ao9eveÎQ oTaôiou I KaTâ NYHA: ôe ouvioTâTai âÀÀo 0âppaKo nÀnv TnQ ßaoiKHQ unooTnpiKTiKHQ aY^YHQ. EvôexopévwQ oto péÀÀov nporaû^ouv oToixeia woTe va xpnoiponoioûvTai Kai avTaYwvi-oTeQ twv unoôoxéwv evôo9nÀivnQ

• Ze ao9eveÎQ oTaôiou II KaTâ NYHA pnopoûv va xo-pnYn9oùv:

a. avTaYwvioTéQ twv unoôoxéwv evôo9nÀivnQ-1 (bosentan oe apxiKH ôôon: 62.5mg x2. Yia 1 pHva pe enaKôÀou9n aû^non oe 125mg x2. Kai ambrisentan 5 mg/npépa) H ß. AvaoToÀeÎQ 0wo0oôieoTepâonQ-5 (sildenafil oe ôôon 20mg x3)

• Ze ao9eveÎQ oTaôiou III KaTâ NYHA pnopoûv va xopnYn9oûv:

a. avTaYwvioTéQ twv unoôoxéwv evôo9nÀivnQ-1 (bosentan H ambrisentan) ß. AvaoToÀeÎQ 0wo0oôieoTepâonQ-5 (sildenafil), Y. AvâÀoYa npooTaKuKÀÎvnQ (eionveôpevn iÀonpo-oTn, unoôopia TpenpooTeviÀn H ouvexHQ evôo0Àé-ßia éYXuon enonpooTevôÀnQ oe apxiKH ôôon: 2 ng/ kg/min).

Ze eiôiKéQ nepinTwoeiQ pe au^npévo Kivôuvo pnopei va Yivei évap^n pe ouvôuaopô eiôiKûv 0appâ-kwv

• Ze ao9eveÎQ oTaôiou VI KaTâ NYHA pnopei va xo-pnYn9ei:

a. ouvexHQ evôo0Àéßla éYxuon enonpooTevôÀnQ oe apxiKH ôôon: 2 ng/kg/min Kai oe eiôiKéQ nepi-nTûoeiQ oe ouvôuaopô pe bosentan, ambrisentan H sildenafil

H xopHYnon avTinnKTiKHQ aYWYHQ oe ao9eveÎQ pe nveupoviKH apTnpiaKH unépTaon oxeTiZôpevn pe to ouoTnpaTiKô oKÀnpôôeppa ôev evôeiKvuTai22.

r. Aidpeon nveupoviKH Ivœon

Aûo KaÀâ oxeôiaopéveQ, TuxaionoinpéveQ, eÀeYxôpe-veQ pe eiKoviKô 0âppaKo peÀéTeQ éôei^av oti n ku-KÀo0wo0apiôn eivai opiaKâ anoTeÀeopaTiKH oTn oTa-9eponoinon H ßeÀтíwon TnQ ôiâpeonQ nveupoviKHQ ivwonQ oe ao9eveÎQ pe ouoTnpaTiKô oKÀnpôôeppa2324. Anô tiq unâpxouoeQ peÀéTeQ npoKûnTei ôti:

• H KuKÀo0wo0apiôn xopnYoûpevn Yia 6-12 pHveQ, eÎTe evôo0ÀeßíwQ oe pnviaieQ woeiQ (600mg/m2/ pHva) 23, eÎTe per os (1-2mg/kg/npépa) 24, éxei 9éon oTn 9epaneia TnQ ôiâpeonQ nveupoviKHQ ivwonQ oto ouoTnpaTiKô oKÀnpôôeppa.

• H KuKÀo0wo0apiôn pnopei va ouYxopnYn9ei pe pe9uÀnpeôviZoÀôvn per os oe ôôoeiQ ewQ 20mg/ npépa 23. Y^nÀôTepeQ ôôoeiQ npenei va ano0eû-YovTai Yia to evôexôpevo enaYWYHQ ve0piraiQ Kpi-onQ.

• MeTâ to népaQ TnQ aYWYHQ pe KuKÀo0wo0apiôn npoTeiveTai wq 9epaneia ouvTHpnonQ n aZa9eio-npivn (2.5mg/kg/npépa, péYioTn ôôon 200mg/npé-pa) 23 H to puKo0aivoÀiKô o^û25.

• Zûp0wva pe nepiopiopeva ß^ioYpa0iKa ôeôo-péva, oe av9eKTiKéQ nepinTûoeiQ pnopei va xpn-oiponoin9ei rituximab26 (otiq eYKeKpipéveQ ôôoeiQ Yia Tnv PeupaToeiôH Ap9pÎTiôa), pe npoooxH ôpwQ oTnv ouYxopHYnon u^nÀwv ôooewv KopTiKoeiôwv, woTe va ano0eux9ei to evôexôpevo ve0piraiQ Kpi-oewQ

A. Ne^piKH Kpion ZKÀnpoôéppaxoç

Ze ve0piKH Kpion oKÀnpoôéppaToQ xopnYoûvTai ava-oToÀeÎQ peTaTpenTiKoû evZûpou aYYeioTevoivnQ (nx. KanTonpiÀn H evaÀanpiÀn) oe u^nÀéQ ôôoeiQ, Ka9ûQ ôûo peYâÀeQ npoonTiKéQ pn TuxaionoinpéveQ peÀéTeQ

of RHEUMATOLOGY 1

E Л Л H N I К H РЕУМАТОЛОпА 2015

éбelÇav oпpavтlкП ßeЛтíwoп тпд eпlßíwoпg Kaí peíwoп тпд aváYкПQ Yía póvlpп alpокá9aрoп pe тп XPПoп au-^v тшv пaрaYÓvтшv27,28.

Era aпотuxíaQ тшv avaoTC^éwv peтaтрeптlкоú evZú-pоu aYYelотevoívпQ va eЛéY^оuv lкavопоlптlкá тп ve-ФР1кп крíoп тои oкЛпробéрpaтоQ бev uпáрxоuv тeк-pпplwpéveg кaтeu9uvтПрleQ обпYÍeQ. Прóoфaтa, pía оpáбa erô^wv aпó тоv Kavaбá прóтelve тпv пpоo9П-кп avaoTC^éwv бlaúЛwv aoßeoтíоu п a^pevepY^wv avтaYшvloтшv29 EпlпÀéоv, uпáрxоuv avaфорéQ то-рloтaтlкшv, 0пои oe veфрlкП крíoп oкЛпpобéppaтоg XорпYП9пкav aпокЛeloтéQ uпобохéwv aYYelотevoívпQ II, Kaí avтaYШVloтéQ uпобохéwv evбо9пЛívпg-130 era-пÀéоv тшv avaorc^éwv peтaтрeптlкоú evZúpоu aYYeí-отevoívпQ. ТеЛод, oe av9eKríKÉQ ^pím^eíc ra9a-vúq Kaí п evбофЛéßla xорПYПoп avaЛóYwv пpоoтaкu-кЛívпQ (iloprost 0.5-2ng/kg/min) va eívaí eпwфeЛПg31.

E. npooßoAn Гaстгpevтeplкоú Zuo^MaTOQ

Oí ouoтáoelç Yía тпv avтlpeтwпloп тпд проoßоЛПç тои Yaoтрevтeрlкоú ouoтПpaтоQ oe ao9eveiç pe ou-oтпpaтlкó oкЛпрóбeрpa oтпрíZоvтal KupíwQ oe av0í-хтед peЛéтeç Kaí oтп Yvwpп тшv etóíKúv, га9шд бev uпáрxоuv б^ед тифЛед тuxalопоlПpéveQ peЛéтeQ. Протeívоvтal тa aкóЛоu9a:

• Oí avaoтоЛeÍQ avтЛíaQ пpwтоvíwv eívaí aпотeЛe-opaтlкоí oтпv avтlpeтшпloп тпд oxeтlZópevпQ pe то ouo^a^^ oкЛпрóбeрpa YaoтрооloофaYlкПQ пaЛlvбрópпoпQ, тпд oтévшoпQ тои 0l00фáY0U Kaí тшv eЛкшv тои 0l00фáY0u32.

• Oí прокмпт^ пaрáY0Vтeд (пх. боpпeрlбóvп 10-20mg qid, peтокЛопрapíбn 10-15mg qid) prop0Úv va хPП0lpопоlп9оúv oтпv avтlpeтшпloп тwv ou-pптшpáтшv тпд Ya0тр00l00фaYlкПд пaЛlvбрópп-oпд, тоu peтeшрlopоú, тпд бuoфaYÍaд, тоu прш-íp0u K0pe0p0ú, тпд фeuбоaпóфpaÇпд32 33.

• H бuoaпоррóфпoп поu офeíЛeтal oe 0úv6p0p0 uпeрaváптu^пд ßaкгпpíwv pпорeí va avтlpeтшпl-o9eí pe кuкЛlкП хорПYП0П avтlßlотlкшv (пх. гаюЛ0-veд, apо^uкlЛЛívп-кЛaßоuЛavlкó, кeфaЛeÇívп-pe-троvlбaZóЛп, тeтрaкuкЛíveд, p^a^íp^) 32.

• Ze oоßaрéд TOplm-woe^ evтeрlкПд проoßоЛПд (бlaтaрaхéд кlvптпкóтптaд, ßaкгпрlaкП urapa-váпгu^п) evбéхeтal va eívaí aпотeЛeopaтlкП п Octreotide (50mg sc^pépa) 32 34.

ZT. npooßoAn Лёрматод

ría тпv №ш0п тоu бéppaтод oe ao9eveíд pe ouo^pa-пк0 oкЛпpóбeppa пpотeíveтal п хРП0П pe9отpe^áтпд (per os п IM) oe б00П 10-20mg/eßбоpáбa35 36. Л0к1-pод eívaí eпíoпд о ouvбuaopóд pe YЛuкокоpтlкоelбП oe хapпЛeд бóoelд (<7,5mg/пpépa).

Z. Аp0píтlбa-OpoYOvíтlбa

Méхpl oтlYpПд бev uпápхоuv бeбоpéva ará ^aí0-

поlПpéveд peЛéтeд Yía ^v avтlpeтшпloп тпд ap9pm-бaд п тпд оpоY0víтlбaд oe ao9eveíд pe ouo^a^^ oкЛпpóбeppa. Epпleplкá хPП0lpопоleíтal ouvбuaopóд pe9отpe^áтпд (per os п IM) oe б00П Ю^й^^^бо-páбa pe хapпЛéд бóoelд YЛuкокоpтlкоelбwv (<7,5mg/ пpépa). ría тпv avтlpeтшпloп тпд ap9píтlбaд éхel хрп-olpопоlп9eí eпíoпд п ЛeфЛоuvоpíбп37 pe кaЛá aпотe-Лéopaтa oe plкpó apí9pó ao9evúv. Oí пepl00óтep0l elбlкоí бe ouvloтоúv тп хоpПYП0П anti-TNF-a пapaYÓ-vтшv шд 9epaпeía тпд ap9píтlбод 0т0 ouo^a^^ oкЛпpóбeppa38.

H. npooßoAn Kapôiaç

Лev uпápхоuv elбlкéд обпYÍeд. AvaЛоYa pe то eíбод тпд пpоoßоЛПд (коЛгакед appu9píeд, пЛпрпд appu9-pía, коЛпокоЛгакод aпокЛelopóд, бlaoтоЛlкП П/кal ouoтоЛlкП бuoЛelтоupYÍa aploтepПд коlЛíaд, loхalplкП puокapбlaкП vóoод, кapбlaкП aveпápкela) eфappó-Zоvтal тa avтíoтоlхa 9epaпeuтlкá пpштóкоЛЛa тпд кapбlоЛоYÍaд

O. EpßoAiaoMoi ото ZKAnpóSepMa

• Лev uпápхel avтévбel^п 0тп X0pПYП0П aбpavопоl-пpévшv, то^lкоYevvшv п epßоЛíшv uпоpоváбшv oe ao9eveíд pe ouo^a^^ oкЛпpóбeppa, поu Лap-ßávоuv avоoокaтaoтaЛтlкП aYWY^ fioTO00 eTOí-бп п avоolaкП кáЛuфп oe auтоúд тсюд ao9eveíд evбéхeтal va uпоЛeíпeтal, prapeí va хpelaoтоúv пeplooóтepeд avapvпoтlкéд бóoelд.

• ^тсюд ao9eveíд pe ouo^a^^ oкЛпpóбeppa, поu Лapßávоuv avоoокaтaoтaЛтlкП aYШYП avтev-бeíкvuvтal тa Zwvra e^ao9evпpéva epßóЛla.

• Eфóoоv aпalтeíтal, о epßоЛlaopóд eívaí проп-póтepо va оЛокЛпpшveтal тоuЛáхloто 3-4 eßбо-páбeд про тпд évap^ тпд avоoокaтaoтоЛПд. EvaЛЛaкгlкá, Zшvтa epßóЛla pпоpоúv va хоpпYП-90úv oтоuд ao9eveíд pe oкЛпpóбeppa поu Лap-ßávоuv avоoокaтaoтоЛП тоuЛáхloто 3 pПveд peтá то пépaд тпд avоoокaтaoтaЛтlкПд aYШYПд, пávтa ouvuпоЛоYÍZоvтaд то eíбод кal тп ßapúтптa тпд Лпф9eíoaд avоoокaтaoтaЛтlкПд aYШYПд.

• TоvíZeтal п oпpaoía tou eтПolоu avтlYplппlкоú ep-ßоЛlaopоú га9шд кal тоu avтlпveupоvlококкlкоú epßоЛlaopоú au^v тшv ao9evúv39

I. Yпooтпplктlкп Mn Фapмaкeuтlкп AYWYP

Me бeбоpévп ^v тepáoтla eпlßápuvoп поu eпlфé-peí то ouoтпpaтlкó oкЛпpóбeppa oтпv кa9пpeplvó-тптa тшv пaoхóvтшv, eппpeáZоvтaд pía oeípá ará пapapéтpоuд 0пшд eívaí п lкavóтптa Yía áoкпoп, п ЛelтоupYlкóтптa тшv хeplwv, п eíráva тоu owpaTC^, п oe^оuaЛlкП ЛelтоupYÍa, о úпvод, п lкavóтптa Yía ep-Yaoía к.a., Yíveтal avтlЛпптП п lбlaíтepп oпpaoía тшv PП фappaкeuтlкшv utoo^i^^^ 9epaTOlüv. Пр0-oфaтa бпpоoleúтпкe pía oeípá aпó обпYÍeд oхeтlкá

pe tiq pn 0appaKeuTiKEQ GepaneuTiKeQ napepPaoeiQ nou npoTeivovTai wq KaTaXXnXeQ Yia touq aoGeveiQ pe oKXnpoSeppa40. Ze aureQ nepiXapPavovTai peTa^u aXXwv:

• QuoioGepaneia/epYoGepaneia Yia Tn PeXTiwon TnQ XeiToupYiKoTnTaQ twv xepiwv.

• OpaSeQ aXXnXoPon9eiaQ- SiKTua unooTiipi^nQ peow TnXe0wvou H SiaSiKTuou Yia ^uxoXoyikh evi-oxuon/PeXTiwon TnQ eiKovaQ tou owpaToQ K.a.

ZuZnTnon-^UMnepaoMaxa

H 9epaneuTiKH avTipeTwnion tou ouoTnpaTiKou oKXnpoSepparoQ exei GeapaTiKH npooSo Ta TeXeuTaia xpovia, eniTpenovTaQ Tnv napepPaon oe eKSnXwoeiQ TnQ vooou, onwQ n ve0piKH Kpion Kai n nveupoviKH apTnpiaKH unepTaon, Yia tiq onoieQ pexpi npo eTwv Sev unHpxe Kapia anoTeXeopaTiKH avTipeTwnion. na-poXa aura, pexpi oqpepa, Sev exei PpeGei 0appaKo nou va Tpononoiei ouvoXiKa Tnv evepYoTnTa Kai e^eXi-^n rou oKXnpoSeppaToQ. Qq eK toutou n npoonaGeia oTpe0eTai oTn PeXTiwon twv oupnTwpaTwv ano Ta enipepouQ opYava otoxouq nou to voonpa nXHTTei. KaGwQ n naGoYeveia tou ouoTnpaTiKou oKXnpoSeppaToQ YiveTai oXo Kai nio KaXuTepa KaTavonTH, veoi 9epaneuTiKoi otoxoi eniXeYovTai ouvexwQ41 pe anw-

Tepo oKono Tnv avanTu^n vewv oKeuaopaTwv nou 9a eivai ao0aXeoTepa Kai anoTeXeopaTiKoTepa ano Ta HSn unapxovTa.

ZTnv napouoa epYaoia eYive npoonaGeia va avaoKo-nnGouv Kai va ouoTnpaTonoinGouv oi TpexouoeQ GepaneuTiKeQ eniXoYeQ Yia to ouoTnpaTiKo oKXnpoSeppa eni Tn Paoei twv ouoTaoewv nou SnpooieuGnKav npoo0aTa ano Tn EULAR-EUSTAR Kai twv anoTeXe-opaTwv eniXeYpevwv peXeTwv. Ze apKeTeQ nepimw-oeiQ nou Sev unapxouv enapKH SeSopeva Yia Tnv TeK-pnpiwon TnQ anoTeXeopaTiKoTnTaQ piaQ GepaneuTi-khq napepPaonQ, napouoiaZeTai n ouvqGnQ npaKTiKq PaoiZopevn oTn Yvwpn twv eiSiKwv. Oi ouoTaoeiQ nou ava0epGnKav anooKonouv povo oTn SieuKoXuvon TnQ Xi^nQ kXivikwv ano0aoewv Kai oe Kapia nepinTwon Sev unoKaGioTouv Tnv Kpion tou kXivikou YiaTpou. ZTnv TeXiKq Siapop0won evoQ GepaneuTiKou oxHpaToQ, eKToQ ano tiq evSei^eiQ Kai avTevSei^eiQ TnQ eKaoToTe GepaneiaQ Ga npenei na-vTa va ouvunoXoYiZovTai Kai aXXeQ napapeTpoi onwQ to kootoq Kai n SiaGeoipoTnTa KaGe GepaneiaQ, oi ouvGqKeQ TnQ Zwhq Kai oi eniGupieQ tou aoGevouQ, Kai KupiwQ n PapuTnTa Kai Ta iSiaiTepa xapaKTnpioTiKa tou vooqpaToQ oe KaGe aoGevq. 42

nivaKaq 1. Ta^ivopriori twv ao8evwv pe nveupoviKH unepTaon Paoei tou AeiToupYiKou oraSiou Kara New York Heart Association (WHO 1998)

AeiroupYiKo oraSio 1 Ao8eveiQ pe nveupoviKH unspraon aAAa xwpiQ ouvaKoAou8o nepiopiopo rnQ iKavornraQ Yia $uoiKr| Spaornpiornra. H ouv|8nQ $uoiKn Spaornpiornra Sev npoKaAei Suonvoia | Konwon, novo oro orr8oQ, | npo-ouYKonriKo eneiooSio

AeiroupYiKo oraSio II Ao8eveiQ pe nveupoviKH unspraon nou ouvenaYerai nepiopiopo rnQ iKavornraQ Yia ^uoiKr Spaornpiornra. XwpiQ evoxAHoeiQ ornv avanauon. H ouv|8nQ ^uoiKr Spaornpiornra npoKaAei Suonvoia | Konwon, novo oro orr8oQ, | npo-ouYKonriKo eneiooSio.

AeiroupYiKo oraSio III Ao8eveiq pe nveupoviKH unspraon nou ouvenaYerai onpavriKo nepiopiopo rnQ iKavornraQ Yia ^uoiKr Spaornpiornra. XwpiQ evoxAroeiQ ornv avanauon. Hniorepn rnQ ouv|8ouQ ^uoiKr Spaornpiornra npoKaAei Suonvoia | Konwon, novo oro orr8oQ, | npo-ouYKonriKo eneiooSio.

AeiroupYiKo oraSio VI Ao8eveiQ pe nveupoviKH unspraon nou aSuvarouv va enireAsoouv onoiaSinore t^uoiKr Spaornpiornra xwpiQ oupnrwpara. Oi ao8eveiQ ep^avi^ouv onpeia Se^iaQ KapSiaKHQ avenapKeiaQ. Auonvoia H/Kai Konwon eKSnAwvovrai aKopn Kai ornv avanauon. OnoiaSnnore ^uoikh Spaornpiornra eniSeivwvei rn oupnrwparoAoYia touq.

of RHEUMATOLOGY 1

EAAHNIKH PEYMATQAOriA 2015

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