www.pediatric-rheumathology.printo.it 12/2003 1 bexCeTis ...

exCeTis daavadebawww.pediatric-rheumathology.printo.itra daavadebaa?bexCeTis sindromi, anu bexCeTis daavadeba (BS) ucnobi mizeziTgamowveuli sistemuri vaskulitia (sisxlZarRvebis anTeba), romelsacaxasiaTebs morecidive oraluri da genitaluri wylulebi; Tvalebis,saxsrebis, kanis, sisxlZarRvebis da nervuli sistemis dazianeba. BSwarmosdgeba Turqi eqimis, prof. hulusi bexCeTis saxelidan, romelmacaRwera es daavadeba 1937 wels.ramdenad xSiria?BS xSirad gvxvdeba msoflios zogierT regionSi. BS-is geografiuliganawileba emTxveva istoriul “abreSumis gzas”. igi ZiriTadadaRwerilia Soreuli aRmosavleTis, Sua aziis da xmelTaSua zRvis auzisqveynebSi, rogoricaa magaliTad iaponia, korea, CineTi, TurqeTi, tunisida maroko. iaponiaSi mozrdil mosaxleobaSi misi sixSirea 1/10 000,TurqeTSi - 1-3/1 000, xolo CrdiloeT evropaSi - 1/ 300 000.SeerTebul Statebsa da avstraliaSi cota SemTxvevaa aRwerili. maRaliriskis mosaxleobaSic ki BS bavSvebSi iSviaTia. 16 wlamde asakispacientebi BS-is yvela SemTxvevis mxolod 3%-s Seadgens. daavadebasaSualod 20-35 wlis asakSi iwyeba. orive sqesis warmomadgenlebSiTanabrad aris gavrcelebuli, Tumca ki mamakacebSi ufro mZimedmimdinareobs.ra aris daavadebis gamomwvevi mizezi?daavadebis mizezi ucnobia (idiopaTiuria). genetikur winaswarganwyobasSesaZloa, garkveuli mniSvneloba hqondes BS-is ganviTarebaSi. raimespecifiuri trigeruli faqtori cnobili ar aris.ramdenime centrSi ikvleven daavadebis mizezebsa da mkurnalobismeTodebs.memkvidreobiT gadaecema?BS-is damemkvidrebis raime konkretuli meqanizmi ar gaaCnia, Tumca kigenetikuri faqtoris arseboba axasiaTebs. sindromi asocirebuliagenetikur winaswarganganwyobasTan (HLA B5), gansakuTrebiT impacientebSi, romlebic warmoSobiT xmelTaSuazRvispireTidan, an Suaaziidan arian. literaturaSi aRwerilia ramdenime ojaxuri SemTxveva.ratom daavadda Cemi Svili? SeiZleba am daavadebis Tavidan acileba?daavadebis mizezi ucnobia. BS-is Tavidan acileba ver xerxdeba.112/2003

gadamdebia?ara, gadamdebi ar aris.rogoria ZiriTadi simptomebi?1) piris Rrus wylulebi (afTebi): es dazianebebi TiTqmis yovelTvisvlindeba. pacientTa daaxloebiT or mesamedSi piris Rrus wylulebidaavadebis pirveli niSnebia. bavSvebis umravlesobas uviTardebamravlobiTi, mcire zomis wylulebi – SeuZlebelia maTi garCevamorecidive wylulebisagan, romlebic xSiria bavSvTa asakSi. didi zomiswylulebi ufro iSviaTia, magram mkurnalobas SeiZleba ufro Zneladeqvemdebarebodes.2) genitaluri wylulebi: vaJebSi wylulebi lokalizebuliaZiriTadad skrotumze da, ufro iSviaTad, penisze. mozrdil mamakacebSiTiTqmis yovelTvis aRiniSneba wylulisSemdgomi nawiburebi. gogonebSiZiriTadad garegani sasqeso organoebi ziandeba. es wylulebi pirisRruSi arsebuli afTebis msgavsia. bavSvebs pubertatis asakamdegenitaluri wylulebi naklebad aReniSnebaT.vaJebSi SeiZleba morecidive orqiti (saTesleebis anTeba) aRiniSnebodes.3) kani: gvxvdeba kanis sxvadasxvagvari dazianeba. aknesmagvaridazianebebi mxolod pubertatis asakis Semdeg aRiniSneba; vlindebakvanZovani eriTema - wiTeli, mtkivneuli, kvanZovani dazianebebi,romlebic upiratesad fexebis qveda nawilis zedapirze mdebareobs.es dazianebebi ufro xSiria pubertatis winare asakis mqone bavSvebSi.paTergiuli reaqcia aris BS-is mqone pacientebis kanis pasuxi nemsiTCxvletaze, rac BS-is sadiagnostiko testia. winamxarze sterilurinemsiT Cxvletis Semdeg 24-48 sT-Si papula an pustula viTardeba.4) Tvalebi: es daavadebis yvelaze mZime gamovlinebaa. simptomissaerTo sixSirea 50%, magram vaJebs Soris es maCvenebeli 70%-mdeizrdeba. gogonebs Tvalebis dazianeba ufro iSviaTad aReniSnebaT.pacientTa umravlesobaSi dazianeba ormxrivia. procesSi TvalebisCarTva, Cveulebriv, sindromis gamovlinebidan pirveli 3 wlisganmavlobaSi xdeba.Tvalebis dazianeba qronikulad, perioduli gamwvavebebiTmimdinareobs. ziandeba Tvalis rogorc wina, ise ukana kamera (wina daukana uveiti). yoveli Setevis Semdeg rCeba garkveuli struqturulidazianeba, rac TandaTanobiT mxedvelobis gauareseba-dakargvas iwvevs.5) saxsrebi: saxsrebi procesSi erTveba BS-iT daavadebul bavSvTa 30-50%-Si. Cveulebriv ziandeba koWebi, muxlebi, majebi da idayvebi.aRiniSneba monoartikuluri (erTi saxsris), an oligoartikuluri (4-zenaklebi saxsris) dazianeba. procesi Cveulebriv, ramdenime kvirasgrZeldeba da ukuganviTarebas ganicdis narCeni movlenebis gareSe. BSisarTrits saxsris dazianeba Zalze iSviaTad Tu mohyveba.212/2003

6) nervuli sistema: nevrologiuri dazianebebi, marTalia, iSviaTad,magram mainc aRiniSneba BS-iT daavadebul bavSvebSi. damaxasiaTebeliagulyrebi da momatebuli qalasSida wneva, romelsac Tan axlavs Tavistkivili da cerebruli simptomebi. yvelaze mZime formebi mamakacebSigvxvdeba. zogierT pacients SeiZleba fsiqiatriuli problemebi gauCndes.7) sisxlZarRvebis dazianeba iuveniluri BS-is dros 12-30%-Sigvxvdeba. igi cudi gamosavlis mimaniSnebelia.Cveulebriv, ziandeba msxvili sisxlZarRvebi. xSiria wvivis venebisdazianeba, rac wvivebis SeSupebasa da tkivils iwvevs.8) kuW-nawlavis dazianeba: is gansakuTrebiT xSiria pacientebSiSoreuli aRmosavleTidan. nawlavebis gamokvleviT wylulebi vlindeba.daavadeba yvela bavSvSi erTnairad mimdinareobs?ara. zogierTs SeiZleba daavadebis msubuqi forma hqondes, piris RruswylulebiT da kanis dazianebebiT, xolo sxvebs ganuviTardes Tvalebis,an nervuli sistemis dazianeba. aseve arsebobs gansxvaveba gogonebsa davaJebs Soris. vaJebSi, Cveulebriv, daavadeba ufro mZimed mimdinareobs,Tvalebisa da sisxlZarRvebis ufro xSiri dazianebiT, vidre gogonebSi.gansxvavdeba Tu ara daavadeba bavSvebsa da mozrdilebSi?BS bavSvebSi mozrdilebTan SedarebiT iSviaTia. pubertats garkveulimniSvneloba aqvs. daavadeba postpubertatuli asakis bavSvebSi ufromozrdilebis msgavsad mimdinareobs. BS-is mqone bavSvebSi ufrosebTanSedarebiT ufro xSiria ojaxuri SemTxvevebi. sazogadad, miuxedavadgarkveuli variaciebisa, BS bavSvebSi da mozrdilebSi msgavsimimdinareobiT xasiaTdeba.rogor diagnostirdeba?diagnozi ZiriTadad klinikur suraTs emyareba. SesaZloa 1-5 weligavides, vidre bavSvis daavadeba BS-is saerTaSorisokriteriumebsDdaakmayofilebdes. Cveulebriv diagnozis dasmas saSualod3 weli sWirdeba.BS-isTvis specifiuuri laboratoriuli monacemebi ar arsebobs.bavSvebis daaxloebiT naxevari HLA B5-is matarebelia, rac daavadebisufro mZime mimdinareobasTan aris dakavSirebuli.rogorc zemoT iTqva, kanis paTergiuli testi dadebiTia pacientTa 60-70%-Si.sisxlZarRvTa da nervuli sistemis dazianebis diagnostikisaTvissaWiroa sisxlZarRvebisa da Tavis tvinis specifiuri vizualizacia.vinaidan BS multisistemuri daavadebaa, pacientebis mkurnalobaSiCarTulni unda iyvnen da ermaneTTan TanamSromlobdnen Tvalis(ofTalmologi), kanisa (dermatologi) da nervuli sistemis(nevrologi) specialistebi.312/2003

a mniSvneloba aqvs gamokvlevebs?1) diagnostikis TvalsazrisiT mniSvnelovania kanis paTergiulitesti. igi Sedis bexCeTis daavadebis Seswavlis saerTaSoriso jgufismier mowodebuli klasifikaciis kriteriumebSi. steriluri nemsiTwinamxris SigniTa zedapirze keTdeba 3-5 naCxvleti, rac mcireodenmtkivneulia. reaqcia fasdeba 24-48 sT-is Semdeg.hiperreaqtiulobis naxva SeiZleba sisxlis aRebis adgilasac, anqirurgiuli Carevis Semdeg, amitom BS-is mqone pacientebi arasaWiroCarevebs unda moeridon.2) diferenciuli diagnostikisaTvis tardeba sisxlis gamokvleva,Tumca BS-isTvis specifiuuri laboratoriuli testi ar arsebobs.maCveneblebi, romlebic anTebaze miuTiTebs, msubuqad aris momatebuli.SesaZloa, aRiniSnos zomieri anemia da leikocitozi. am gamokvlevebisgameoreba saWiro ar aris, Tu pacients ar utardeba monitorirebadaavadebis aqtiurobis, an medikamentebis gverdiTi moqmedebisgansazRvris mizniT.3) sisxlZarRvovani da nevrologiuri dazianebis mqone bavSvebSimowodebulia vizualizaciis ramdenime meTodi.ikurneba Tu ara?daavadebas axasiaTebs remisia, magram mosalodnelia Semdgomigamwvavebebi. daavadebis kontroli SesaZlebelia, gankurneba - ara.rogoria mkurnaloba?vinaidan BS-is mizezi ucnobia, misi specifiuuri mkurnaloba ar arsebobs.sxvadasxva organos dazianebis SemTxvevaSi arsebobs alternatiulisamkurnalo meTodebis farTo speqtri. am speqtris erT mxares BS-ismqone is pacientebia, romelTac saerTod ar esaWiroebaT mkurnaloba,meore mxares ki pacientebia Tvalebis, centraluri nervuli sistemisa dasisxlZarRvebis dazianebiT, romelTac kompleqsuri mkurnalobasWirdebaT.BS-is mkurnalobis Sesaxeb arsebuli TiTqmis yvela monacemimozrdilebze Catarebuli kvlevebiT aris miRebuli. qvemoTCamoTvlilia ZiriTadi wamlebi:a) kolxicini: uwin es preparati gamoiyeneboda BS-is TiTqmis yvelagamovlinebis dros, magram bolodroindelma kvlevam aCvena, rom igiufro efeqturia saxsrovani problemebisa da kvanZovani eriTemissamkurnalod.b) kortikosteroidebi: isini Zalze efeqturia anTebis samkurnalod.steroidebi ZiriTadad eniSnebaT Tvalebis, cns-isa da sisxlZarRvebisdazianebis mqone bavSvebs, Cveulebriv, didi dozebiT, peroralurad (1-2mg/kg dReSi). saWiroebis SemTxvevaSi SesaZlebelia maTi intravenuriSeyvana maRali doziT (30 mg/kg 3 dReSi erTxel), raTa miRweul iqnas412/2003

swrafi Sedegi (puls-Terapia steroidebiT). piris Rrus wylulebisa daTvalis dazianebis samkurnalod steroidebi gamoiyeneba adgilobrivad.g) imunodepresantebi: am jgufis preparatebi bavSvebs eniSnebaTdaavadebis mZime mimdinareobis dros, gansakuTrebiT Tvalebisa daSinagani organoebis dazianebisas. es preparatebia: azaTioprini,ciklosporin-A da ciklofosfamidi.d) antiagregantuli da antikoagulanturi Terapia: gamoiyenebazogierT SemTxvevaSi sisxlZarRvebis dazianebis dros. pacientebisumravlesobaSi am mizniT aspirinis gamoyeneba sakmarisia.e) piris Rrus da genitaluri wylulebis adgilobrivi mkurnaloba.v) anti-TNF mkurnaloba: preparatebis es axali jgufi specialurcentrebSi gadis Sefasebas.z) Talidomidi gamoiyeneba zogierT centrSi piris Rrus didi zomiswylulebis samkurnalod.eqimebis mier BS-is mkurnaloba da dakvirveba erToblivad undaxdebodes. specialistTa gundSi pediatri revmatologis garda undaSediodnen ofTalmologi da hematologi. ojaxi, an Tavad pacientimudmiv kontaqtSi unda iyvnen maT mkurnalobaze pasuxismgebel eqimTan,an samkurnalo dawesebulebasTan.rogoria medikamenturi mkurnalobis gverdiTi moqmedeba?1) diarea kolxicinis yvelaze xSiri gverdiTi efeqtia. iSviaTSemTxvevebSi am preparats SeuZlia gamoiwvios leikocitebis, anTrombocitebis ricxvis Semcireba. aris monacemebi azoospermiaze(spermatozoidebis raodenobis Semcireba), magram Terapiuli dozebisSemTxvevaSi es problema ar iqmneba.2) kortikosteroidebi yvelaze efeqturi antianTebiTi preparatebia,Tumca, maTi gamoyeneba SezRudulia xangrZliv gamoyenebasTandakavSirebuli gverdiTi efeqtebis gamo, rogoricaa magaliTad Saqrianidiabeti, hipertenzia, osteoporozi, kataraqta da zrdis Seferxeba.bavSvebSi steroidebis miRebis aucileblobis SemTxvevaSi, TuSesaZlebelia, es preparatebi unda dainiSnos erTjeradi dilis dozissaxiT. xangrZlivad miRebisas damatebiT unda dainiSnos kalciumispreparatebi.3) imunodepresantebi: azaTioprini SeiZleba hepatotoqsiuri iyos(toqsiuri RviZlis mimarT), Seamciros sisxlis formiani elementebisraodenoba da gazardos mgrZnobeloba infeqciebisadmi. ciklosporin-AZiriTadad Tirkmlebis mimarT toqsiurobiT xasiaTdeba da SeiZlebahipertenzia gamoiwvios. aseve SesaZloa, aRiniSnos Tmianobis momatebada RrZilebis dazianeba. ciklofosfamidis ZiriTadi gverdiTimovlenebia Zvlis tvinis funqciis daTrgunva da moqmedeba SardisbuStze. xangrZlivi gamoyeneba menstrualur cikls arRvevs da SeiZlebaunayofoba gamoiwvios. am preparatebis miRebisas pacientebi unda iyvnen512/2003

mkacri dakvirvebis qveS da yovelTviurad, an or TveSi erTxel undaCautardeT sisxlis da Sardis gamokvleva.ramden xans unda grZeldebodes mkurnaloba?am kiTxvaze standartuli pasuxi ar arsebobs. saerTod, imunosupresiulimkurnaloba wydeba, minimum, 2 wlis Semdeg, an roca pacients 2-wlianiremisia aqvs.Tumca ki, sisxlZarRvebisa da Tvalebis dazianebis mqone bavSvebSi,romlebSic sruli remisiis miRweva advili ar aris, mkurnalobaSesaZloa mTeli sicocxle gagrZeldes. aseT SemTxvevaSi medikamentisada misi dozis Secvla klinikuri suraTis safuZvelze unda moxdes.ra SeiZleba iTqvas aratradiciuli/alternatiuli mkurnalobis Sesaxeb?BS-isaTvis aseTi mkurnaloba ar arsebobs.ra saxis perioduli kontrolia aucilebeli?perioduli kontroli aucilebelia daavadebis aqtiurobisa damkurnalobis monitorirebisaTvis da gansakuTrebiT mniSvnelovaniaTvalebis anTebis mqone bavSvebisaTvis. Tvalebis gamokvleva undamoxdes Tvalis specialistis mier, romelic gamocdilia uveitebismkurnalobaSi. sakontrolo gamokvlevebis sixSire damokidebuliadaavadebis aqtivobaze da gamoyenebul medikamentebze.ramden xans grZeldeba daavadeba?Cveulebriv, daavadeba remisiebisa da gamwvavebebis periodebismonacvleobiT mimdinareobs. rogorc wesi, droTa ganmavlobaSi saerToaqtiuroba mcirdeba.rogoria daavadebis grZelvadiani evolucia (prognozi)?ar arsebobs sakmarisi monacemebi BS-is mqone bavSvebis mkurnalobisSemdgomi mdgomareobis Sesaxeb. arsebuli monacemebis mixedviT viciT,rom arsebobs mravali BS-iT daavadebuli, romelsac mkurnalobasaerTod ar esaWiroeba.Tumca ki, bavSvebs Tvalebis, nervuli sistemisa da sisxlZarRvebisdazianebiT specialuri mkurnaloba da dakvirveba sWirdebaT. patarabiWebSi daavadeba ramdenadme ufro mZimed mimdinareobs, vidregogonebSi. Tvalebi daavadebis dawyebidan pirveli wlis ganmavlobaSiziandeba.iSviaT SemTxvevebSi, BS SesaZloa, fatalurad damTavrdes, rasac adgiliaqvs, ZiriTadad, sisxlZarRvebis (filtvis arteriis, an sxvaperiferiuli arteriis anevrizmis gaskdoma), an centraluri nervulisistemis mZime dazianebis dros, nawlavis wylulebis arsebobisas maTiperforaciis SemTxvevaSi, rac gansakuTrebiT xSiria zogierTi eTnikurijgufis warmomadgenel pacientebSi (iaponelebi).612/2003

problemis arsebobis (avadobis) xangrZlivobis ZiriTadi mizeziaTvalebis dazianeba, rac, SeiZleba, Zalze mZimed iyos gamoxatuli.SeiZleba adgili hqondes bavSvis zrdis Seferxebas, rac ZiriTadadmeoradia da steroidebiT mkurnalobasTan aris dakavSirebuli.SesaZlebelia Tu ara sruli gamojanmrTeleba?zogierT msubuq SemTxvevaSi SesaZlebelia, magram umravles SemTxvevaSiSeiZleba aRiniSnebodes mxolod remisiis xangrZlivi periodebi.rogor zegavlenas axdens daavadeba bavSvisa da misi ojaxisyoveldRiur cxovrebaze?yvela sxva qronikuli daavadebis msgavsad, BS gavlenas axdens bavSvisada misi ojaxis yoveldRiur cxovrebaze. Tu daavadebas msubuqimimdinareoba aqvs, Tvalebisa da sxva mniSvnelovani organoebisdazianebis gareSe, ojaxs SeuZlia Cveulebrivi cxovrebiT icxovros.aseT jgufSi yvelaze xSiri problemaa piris Rrus morecidivewylulebi, ras bevri bavSvisTvis aris Semawuxebeli. es dazianebebiSeiZleba mtkivneuli iyos da xels uSlides Wamisa da smis process.Tvalebis dazianeba SeiZleba aseve seriozuli problema Seuqmnas ojaxs.ra SeiZleba iTqvas skolis Sesaxeb?qronikuli daavadebis mqone bavSvebSi swavlis gagrZeleba aucilebelia.BS-is dros Tu ar aris Tvalis, an sxva mniSvnelovani organosdazianeba, BS-iT daavadebul bavSvs skolaSi regularulad SeuZliasiaruli. mxedvelobis daqveiTebisas SeiZleba aucilebeli gaxdesspecialuri saswavlo programebis gamoyeneba.ra SeiZleba iTqvas sportis Sesaxeb?Tu mxolod kani da lorwovania dazianebuli, bavSvs SeuZlia sportulRonisZiebebSi monawileoba. saxsrebSi anTebiTi procesis gamwvavebisdros sports unda movaridoT. arTriti BS-is dros xanmoklea da srulukuganviTarebas ganicdis. anTebis Cacxrobis Semdeg bavSvs SeuZliasports daubrundes.Tvalebisa da sisxlZarRvebis dazianebis SemTxvevaSi bavSvis aqtiurobaunda SeizRudos. pacientebi qveda kidurebis sisxlZarRvebis dazianebiTunda moeridon xangrZlivad fexze dgomas.ra SeiZleba iTqvas dietis Sesaxeb?sakvebis miRebasTan dakavSirebiT aranairi SezRudva arsebobs.axdens Tu ara klimati zegavlenas daavadebis mimdinareobaze?ara. klimatis raime zegavlena BS-is gamovlinebaze ar aris cnobili.SeiZleba bavSvs vaqcinacia Cautardes?712/2003

eqimma unda gadawyvitos, Tu romeli vaqcinis miRebaa bavSvisTvisSesaZlebeli. Tu pacienti imunosupresiul preparats iRebs (steroidebi,azaTioprini, ciklosporin-A, ciklofosfamidi, anti-TNF da sxv.),vaqcinacia cocxali, atenuirebuli virusiT (wiTuris, wiTelas, ybayurasanda sabinis poliomyelitis sawinaaRmdego vaqcinebi) unda gadaidos.im vaqcinebis Seyvana ki, romlebic ar Seicaven cocxal virusebs, aramedmxolod infeqciis gamomwvev proteinebs (hepatit B-s, yivanaxvelas,pnevmokokis, hemofilusis, meningokokis, tetanusis, difteriis, salkispoliomielitis sawinaaRmdego vaqcinebi) - SesaZlebelia.ra SeiZleba iTqvas sqesobriv cxovrebaze, orsulobaze, kontracefciaze?sqesobriv cxovrebasTan dakavSirebuli umTavresi problemagenitaluri wylulebis arsebobaa. isini SeiZleba iyos morecidive,mtkivneuli da, amdenad, xels uSlis sqesobriv cxovrebas. imis gamo, romqalebSi BS-is mimdinareoba metwilad msubuqia, maTi orsuloba SeiZlebanormalurad mimdinareobdes.Tu pacienti imunodepresantebs iRebs, SeiZleba daWirdebskontraceftiuli saSualebebis gamoyeneba. Sesabamisad amisa,kontracefciis warmoebisaTvisa da orsulobis SemTxvevaSi TavianTeqims unda mimarTon konsultaciisaTvis.812/2003