400 / YaeOuuk no neguampua 3ABOJIABAHHA HA CbEJIMHUTEJIHATA ThKAH 11 Boseenne 3aGosiBANHIsITA OT PEBMATHINHS KpBr ce onpesesst KaTO CHCTeEMHM, BRaTATHTe THM Hc HenanecTHa eTHOTOrHs. C deGpit- era H HapymenisTa OT OGL XapaKTep Te HanloxoGaBat HUeKUHOSHO 3abo.sBaHe, HO He Ce HOBANABAT OT NPOTHBOMHeK UO3HO sleueHIte. AAT PAINT MMYHHM HapyLNeHiis 1 pH act OT TAX (T. Hap. KOAreHOSH) avroumynen THN B natorenesata 4M aAe- rowsiMa 4 Te ca oT Bosnamresuure 1 rekanHitre AecTpyK- TBH HAMCHCHHA ca MeAHMpanit OT MMyH- H/WaK OF HM KOMIUTeKeH UNTOTOKCHMHH T-auspounrn KPBBOHOCHITe CbAOBE HMYHHItTe KOMILICK- cH akTHBHpat XeMOTAKCHUMHTe arcHTH H yaouabat ChaoBHA nepMeaduanTeT 3a BBSIKVINTEAMHTE KICTKH, AKTHBHpaT KOM- IuiemenTapnara ceTeMa, pH y4acrHeTo Ha KOsITO Ce PeaINsHpar AccTpykTHBHITE HaMenenits AKTHBHpanero Ha asropeaxtupiire T 1 Banscbountrit © 1pBHMHOTO HMyHOA0- THO Hapylienue pH cHeTEMMHTe ABO OmioxcHH B TbKAHHTe 1 UMYyHHit 3a60BAHIA, NPOBOKIpAHO B Pe AMMA cayYaH OT Bce OMe HeH3sicHeHH BHU H BBTPeUIHH cbaktopH. HLA an- THPeHHTe Ca CAHH OT Hait-chiuecTBEHHTe BETPeUHH dakTOpH Haynnnst, pect. asroumynnnst otro. BOP € CIOHO CLOTHE, B _KOETO HMYHO UNTHTe BIaNMOAeitCTBAT Hpes CpHsHYECKHt KONTAKT KAeTKa C KAeTKA H Npes Mpexka OT pasTHOPHMHt MpoayKTH LNTOKHHH, tpe- HaCAMLM CTHMYAMpAMH WI HUXHOMpAUUt curnat Yrounenn ca acounaunn ¢ onpeseac nit HLA anturenit He camo Ha orneanitre HOSOAOFUMHH CHM, HOH Ha TeXHitTe KOM cyOTHIMIORE € pasta EBOITIO UUs, KOCTO UMa 3HANEHHE 3a ONpesteAAHE Ha mporHosara YrownsaBaneTO Ha MHAMBHAYAIHOTO UMYHONOFHYHO HapyuleHHe Ht reHeTHN- Ha MIpeaNCHOsHUA IPH BeAKO OF peB MaTHMHHTe 3aGOrABAHH AaBA MHKpoOp- Mauna 3a H3G0pa Ha Hait-ateKBaTHOTO aeuenite. 11.1. lOpennsen xponnuen aptpat JOnennzen xponnuen aprput (IOXA) ¢ OGOGMABALO HAHMcHOBAHHe Ha xXeTepo- rena rpyna 3a601sBaHHA, 4MitTO OCHO- BCH CHMITOM © XPOHMYCH CHHOBHT Ht CHI Ho ce pasIM4anar NO M3BbHCTARHHTe cH HposBH, NpoOTHYaHe N1porHosa. Kaacudukauna 4 AMarnocTH4Hn KpHTe- pan. Xponmanusrt aprpu y meuata cBor- BeTCTBA Ha PeBMaTOHAHMs apTpHT ¥ Bb3- PACTHH M10 CBOHTE ABHH CHMITOMIE HL Xa paktepHa eBomouns (eposnH, ysypH, Ac chopMaunn, KOHTpakTypH, aHKHI03It), HO ce OTAMMHBa To HayaTHitTe cH possi, Or- TYK NPOUSTHMAT AMArHOCTHYHHTe, KACH- qbuKaunonnitre 1 TepMAHOTOFNGHHTE pas- sumsnst (TaG. 11.1).3aGonsBanus Ha ceeguiumeanama mokan / 401 Tada. 11 Aluaenocmunnu Kpumepuu na FOXA (EULAR, 1977) Buspact 8 Hayatoro < 16 roaunn ApTpHT B caHa HAM noBeYye cTaBit Mpoanrxnreanoct wa Gonectra 2 3 Meceua (6 ceaMMUM 3a nomHaprpHT) ®opma Ha aprpitra, onpeaenena Ha 6-H MeceuL OT HavamoTO Ha GonecTra a. ommroaprpurna: < 4 crasu 6. noxuaprpurna: 2 5 crap | — ceponerarupna, IgM-pesmaromten cbakrop HeraTHpna — ceponosutuBia, IgM-pessatioaen daktop nosutHBHa (=IOPA)* | B. CHCTEMHa: apTpHT ¢ XapakTepen cbeGpwmTer | 5. Apyrit sa6onapanns, npormuaum © xpormien aprpHT > ionenitteH ankHaosupaut | CNOHAMAOAPTPHT, JOBCHHACH NCOpHATH4eN apPTPHT, BLINAAHTeEAHM YpeBI saGonKBanNs | (yauepo3en Koaut, 6onect Ha Crohn) + TepMtitirsr WOREHNAEH peRMATONLAEN aprpHT ce HH3110.TIHA CAMO MPH apTpHT ¢ HlosiKTHRBEH tect 3a IgM-penmatonaen cbaxtop Ennaemnosorna. Yectotara ¢ 69—196 Ha 100 000 aeua, npw 3a60xenaemoct oKO0 1/10 000 aeua roanuno. Hayanoro ¢ cca 6 Meceuna wespact, ¢ Ba Muka Ha 3a60.1e- Baemoct — I1—3 n 8—12 roa.; choTHoUIe- HMeTO MomMuera: MomueTa ¢ EpeaHo 2:1 (ta6a- TT.2). Ernoaormata ¢ uen3nectita. FponoKit- pau daktopit ca: undpeKuna (rubcola, EBY, parvovirus B 19, cytomegalovirus, herpesviridae, HTLV type I, mycoplasma, Oakrepuannn nentHaorimkann), aBTouMy- HitTeT, TpaBMa, crpec, pH onpescacHa re HeTH4Ha npeaucnosnunsa (Taba. 11.2). Tlavorenesara ¢ asroumyHva, ¢ ywac~ THE Ha MMYHOKOMILICKCHH HM KICTBYHO- McAMMpaHH Mexannamu. Hen3pectHnst cTHOOFMYCH CTHMy.1 © yYacTHeTO Ha cHt- HOBMaTHMTe Makpocparn (cHHOBKOUNTH A) MH ACHAPHTHHTe KIETKH BOIL 10 AKTHBH- pane Ha T- 4 B-kaerknte 4 JoKaHa CHH- Te3a Ha MMyHOPOOYAMHM, BKA. Ha peB- MaTOMAHH chakropH (P@-aBToanTHTAAO cpemy Fe-dyparmenra na IgG), y4acrBa 1M BLB CPOpMUpaHeTO Ha MMYHHM KOMII~ aekew. AKTHBHpaHeTo Ha CHHOBHAIHHTe Makpodarn HM HeyTpoMAHHTe ACBKOLMTH B CHHOBMAIHATA TEMHOCT BOAM 10 TAXHATA Aerpanysauns, OcBoGOACHHTE AN3O3OMHK HSMM, AKTHBMpaHeTo Ha KOMIUIEMeHTap- Hata cHcTeMa, NpoayKUMsTa Ha CBOGOAHH PAAHKAI, Ha BAIOAKTHBHH KHIM, aK- THRAUMSTA Ha PHOpHHOANZATA H CBCHp- BalllaTa CHCTEMA BOJLIT LO HHTCH3HBHO Bb3- nanenne. JIW3030MHHITe NpoTeasHt XHApO- MIMPAT NPOTeOMMMKAMHTE H KONAreHa Ha rapist Xpyuuia ¢ MmocacaBanto OGpasy- Bane Ha HOBM aBroanTurcHH. Tocacapa Tpoamdepaunsa Ha CHHOBHATHH KICTKH HL hopupane Ha rpanysanmMoHna TbKAH, T Hap. nanye, KOIITO 4Pe3 OKAIIHO OCBOGO- Aabane Ha MPOTCOANTHHE CHSHMH CbILO yapexuta Xpyuisuta 1 cyOXOHApaTHaTa KOCT Onpeserena poan B naTorenesaTa Mar unrepaeskunure — IL-1, IL-2, IL-6, TNFa, IFNy, GM-CSF. Kanuwuna kaptana, OcHopnara KH- MHUHa H3IBAa © apmpum, NposBABaLL CCC XapakTepHHTe CHMNTOMH Ha BR3IMAICHHe — oTok, epHTeM, TOIIMHa, 6omKa 1 Orpa- huyena dbyHKus. OTOKBT ¢ pesyaTaT Ha elem Ha MepHaprikyapHuTe MCKH ThKa- HM, BBTPecTABeH HAINB HOT NITICPTPO- dbus ua cHnopnasnara MemOpar sacara Nn BH, & ManKUTe CTABM Ha KHTKHTE.M CTbIa- lara ce 3acsirat MpeAMMHO TIpH MosMapT- pitrHara cpopma. ApTpHT & anodHaeanHt- TE CTABN Ha (TPbOHAMHHA CTBAG KAMHIHO poritia ¢ GOIKH H cKOBAHOCT 8 rHpOa H ire cTa-402 / VueGnuk no nequampua 6a. 11.2 Napaxmepucmuka na IOXA I Tugasiene Cress Hoanaprpirita bopma Oauroaptputna dpopwa | hopwa ceponerarupna [ceponosmtumna | LtHn Iran | Mecrora 10% 30% 10% 30% 20% bpowt | sacernaT | eran | sapnaouaen | > 5 25 <4 <4 | i = Crornowenne no nox won 2M 2>>>M 2>>>M KeeM Buspact # usiaoro aererso | nx 1-3 r >>? <“6r >10 Fr Aptpitr 60% noanapr- | cumerpuyen, | cumerpayen, |acumetpu-lacumetpH- | pur 40% oa | roaesm w maa-| roremt | wen,roaesn | encaxporan- | | roaprpitr kit cramtt Maakit crap | crap, Mowo- | aunt it 11a 02 | aprpur w S0- | irre xpaitiut 10% uit crapienre sonaTHa Yee, I 5% ©) ~20-40% xpo- |~10%ocTEp upnaoumxaur vaeten Lan xponment | Hato © oO (+) 9 100% |) © ANA © (+) 8 25% (+) 75% — | (+) 75-85% | paaxo (+) | HLA DR4, DRS, | DRS, DPw3, | DR4 DRS, DRO, | B27 8 50-75% | acouMauna DRS DQw4 DRS Mportiosa na | 8 40% — ao6pa | osapasspane c| xponnuno: ao6pa mpexoa 6 al aprpira (pu oanroapt- | aecopwaunn | nepenern- kuaAo3Mpau pur). 35% — pau, cnonaiiio apt- osapansinane € ‘aectpyxTH- pur mpi act dhopsauiti,s went or naumennitre % — neosa- pansinamt, aect- pyxritmen Bpara, OFPaHMYCHH BIDKCHHA, AHKHIO3a. EanocrpanHoro HM 3acarane BOAM 20 TOp- THKOJHE H CKONMO3a. 3acsiraHeTo Ha TeM~ NopomanAMOyslapunTe cTaBH € CHMETPHY- HO H BOAM B CROJONMS AO MUKporHaTHs. ApTput Ha KpikoapHTeHouaHnte cTaBH ce YeTaHOBsBA PLLKO HM NIpOTHYa C KIMHHKA Ha JapMnreasen ctpuaop. Mo-paKko e 3a- caranero Ha 6ypcume — vanp. Be fossa poplitea (7. nap. Baker's cyst), 6paxnamna- ra CHHOBHATHA KHCTA H Ip. H3pa3 Ha me- HOCUHOGUM a CKCTCHIOPHHTE CYXOKMINST ca CHHOBHAIHHTe KHCTH NO AOpsaTHata NOBLPXHOCT Ha KHTKHTe H crbratara. Cre- HOSHpallL TEHOCHHOBHT Ha (beKcopHHtTe CYXOKIIMA Ha TIPBCTHTe Ha pbucTe BON O OrTpaHH4eHa CKCTEHSHA H LeopMaLnst 11.1.1. Guctemua dopa (Ganect ua sin) aa Tipu 10% or acuara Hayanoro Ha LOXA. cbc CHCTeEMHH NiposBH. Te Morar Aa nipel-ulecTBaT aprpHTa cBe CCAMMUM, MeceUH H roamnn (ra6a. 11.2) Jmarnocrusinn kpitrepun ca: xapaxrepen: (beOpranter, oOpun, cranen cHHApoM (at- put/aprpaarun). Pedpuaumem (x 100%) € MHTepMutTHpaut (cenTHYeH) xapaKtep 1—2 nua B acHonoumero, Han 39°C; npo- TBLKHTeAHOCT 2 HW NoBeYe ceaMMUM. Ko- wen obpue (8 95%) — aneKpeTHH, ep TMH MaKY.IM, ¢ AMaMeTBp 2—5 mm, pa3- NOAOXCHH MPeAMMHO M0 TAIOTO, NPOKCH MIHHTe YaCTH Ha KpaitHMUNTe Ht IMUCTO, Obp3onpexozeH, NosBABaU ce lO Bpeme Ha Temmepatypen nux. Jpyrn cueremnn NposaBH ca xenamocnrenomecaaua (85%), jumpadenonamua (70%), nepuxapdum (35%), nonaKora ce4eTaH ¢ muokapdum naespum (20%) w nyamonum, Kopemna 60a ka (10%), muareuu. JTaGoparopuara naxoaKa ¢ Hiccrieupi- Ha — anemus, 1eakoumTosa (>15 000/ mm), oneBABAHE H NOmMHYKICO3a, TPOMGOUNTO- sa, yaeamuena CYE u CRP, xunepram robyannemua. 1gM-P® w antianyKncap uit anTireaa (ANA) ca Merarmpnit 11.1.2. Toanaprpurua dopma AlecpHunpa ce Kato aprpuT BS 1 MoBede craBv npes mpsite 6 Meceua Ha GoxtectTa. APIPHTbT € CHMCTpHYCH, NPCAMMHO Ha ro- ICMHTe CTAB — KOJICHHM, TPHBHEHM, J1a- KeTHH H resent, Aprpit B MeTakaprio- 1. MeTarapsodalanreatnn, NpoKCHMaNHit Ht JUMCTAIHM MHTepialanreasiHH CTaBH ce yC- TaHOBABA B OKONO 20—25% (cur. 11.1) Lniinuar rpp6nax 1 TemniopomarnGysiap- HMTe CTaBH Ca HeCTO 3acerHaTH, TeHOCHHO- BuT HMa B 1S—20%. CuctemHiTe npoaBH ca 1O-peaku — HeTHNMYeH deOpHtTeT (30%), xenatocricHomeranna (10%), s1M- (paaenonarus (5%), nepuKxapaur (5%), ruteB- pT H Kopemna Gouxa (1%). Xponmucn yre- HT ce ycraHonaBa B OKONO 5%. Pasamuanar ce Ane bopaut Ha nlonMapt- pur + ceponeratupna, IgM-pesmatomsen JoasBaHus Ha cbegurumenrama mokan 403 Ha JOXA, ur. 11.1. floanaprpumia doops Ma muTepdaranreanutre crap Ha (baxtop HeratuBHa it + ceponosnTHBHa, IgM-permaronacn haxrop nosHTHBHa, aeduNMpana KaTO sosenusen peemamouden apmpum (1OPA) CeponosnTuanata noanaprpitna dbop- sa (IOPA) Hario06ana penmaroutns ap TPHT HIpH BESpACTHHTe — Ha4taro pH 10- roueMH Aca, OPpPs0pasBHBALH Ce CposHit HaIMune Ha pepMaTonAN wbsan,lgM-PD HLA-DR4 (+) (ra6a. 11.2) (+), 11.1.3. Oanroaprputua cbopma Aedsnmpa ce Kato aprput B 4-H 10- MAIKO CTaBH Mpe3 MmbpBHTe 6 Mecela Ha Gonecrra. Ha6monava ce mp oKo10 50% or aenata ¢ IOXA. Aptpirsr ¢ acuerpit- yen, Hail-4ecTO B KONIEHHH, JakeTHit H TIC 3CHHH CTaBH, KaTO B OKOMO 50% ce Kacae sa MOHOApTpHT (B 75% OT TAX — MOHO- aprpit Ha KonsiHHa crana), Pasnapat ce 2 tuna onnroaprpu (ra6.. 11.2): « [7m — npeamano mpi Mosier panita sleTcKa BBIPACT, ACHMCTPHYCH apT- PHT B rosleMH CTaBH, HeCTO CBIIpOBOAILAH ¢ XpoHMMeH Mpesten yBenT; + IL Tun — npeaHMHo np no-ronesi Momuera, MOHO- IIH O1HTOApTPHT Ha TO- JICMM CTaBH Ha AOAHMTe KpatHHit (BK jax06euIpena M cakpomTtaNna), cHTC30NNa~ qua, Yacr of ciyvantre esomonpat B alt KivlosMpaul CnoHsMUIOApTPHT. XapakrepHa ocoGeHoct ¢ BUCOKHAT404 / Vue6nuk no nequampus hpoucnT Ha acua ¢ noroKHTeANH ANA 4 o'NO MlopaxcHe, 3acaraneTo Ha ouNTe — XPOHMMCH yREHT/MpHAOUMKANT: B 65% apycTpanen, ap 5—10% npeawiectsa KaM- Hitinata H39Ka Ha aprpitta. YecTH ca ye~ TOXHCHMSATA — CHHEXHM, AeHTOBILAHA Ke- paronaritt, KaTapakta, maykoa. B 70% Osupansbanero ¢ sedeKTHo — ocacnsBane 8 10-25%. Jleua ¢ BucOK pHeK 3a O4HO nopaxcuite ca: MoMutera, B3pacT < 6 ron, oanroaptpat, ANA (+)yHEA-DRS JUnpepenumaana anarnosa. Bx. “Cra- BH CHHLIpOM pit Apyrit 3aGonsBaHHa” Jlesennero ¢ KommiekcHo. Meduxamen- mosNOMO Aeuenue 3a!\OUBA C_Mecmepoudne npomusosesnaaumeanu cpedcmea (HCIIBC) Aspirin — 80—100 mg/kg/24h, Diglofenac 2—3 mg/kg/24h, Indomethacin — 2‘mg/ kg/24h, Naproxen — 13—20 mg/kg/24h, Ibuprofen — 20—40 mg/ke/ 24h, Tolmetin 25—30 mg/kg/24h, Piroxicam — 0,3— 0,6 me/kg/24h. [pu aeua ¢ nomnaprpr H/MIM CHCTEMHH NpoABH H NpH HeOGpO nosimssane or HCIIBC ce m3noaspar T. Hap. Oasnodedcmeauu anmupesmamuyu. hydroxychroloquin (Resochin) — 5—6 mg/ kg/24h; snari com — 0,7—1 mg/kg/cea- MHMHO MYCKYIINO 3a 6 Mecelia, Nepopalo snato (Auranofin) — 0,1—0,2 mg/kg/24h; D-penicillamin — 10 mg/kg/24h; Sul- fasalazin — 40—S0 mg/kg/ 24h. Hsnoa3- anim uatynocynpecopu ca Methotrexat — 5— 10 mg/m?/ceammuno, Azathioprin — 2— 3,5 mg/ke/24h, Eauxoxopmuxocmepoudume (caMOCTOATeHO WIM B KOMOHHAUHS) ca NloKa3aHM fIpH cHcreMHata cbopMa, xpo- HMMCH YBeHT H TEKLK NoMMapTpHT, HenoB- “Maal, Ce OT ropeHsGpoenHTe MemHKa- Ment B 21032 0,25—1 mg/kg/24h win nysc- Teparua c MeTInpeaNH3o10n — 10-30 mg/kg iv. 3a 1—3 anu, Haynomodyaupa- mu cpeactsa — Cyclosporin (4—6 mg/kg/ 24h), umyHorno6yann (400 mg/kg iv. B 4 aun). Thymopoetin-rpancdep dbaxtop 1 TLlasMatpepesa Ce M3NOM3ZBAT CpaBHUTeEHO psutko. Jlokaano aexenue — werpectasio MEDKCKTHpane Ha LenOKOPTHKOCTepoHHH Mperiapatu, XHMMMeCKa CHHOBEKTOMHA, AOKAIHO NpuOKcHHe Ha HCITBC (resto- Be, YHPBCHTH), KpHoTepanna, pu3HoTepa- Ns, KHHe3HTepanna, OrpanHucna ABitra- reaHa akTHBHoct. Opmoneduano u xupyp- 2UYNO AeMenue — KOPUFUpAULH WHAM, Cres KH, ApTpockoricka Ht onepaTHBHa CHHOBeK- TOMHA, KOperpauint onepaunit, eHOMpO- resupane. Meuxocouuasnama adanmauus npw aeuata c OXA e MHoro BaxKHa. 11.1.4. Peakrusun aprpuTn Peaxtusunte aptputi (PA) ca acenri4- HH, CTCPHAHH apTpHTH, pasBHBaUIN ce B XOula WIM CKOpO Ces pecriMpaTopHa, upeB- Ha WM yporenuTatHa HAdeKuns. Taxnia- Ta NPOABMKUTEAHOCT € OT HAKOIKO eZ MHL O HAKOAKO Mecella HM HMaT 106pa nporHosa Enuazemnosorna. Peakrusen aprpir ce Ha6mozaza cpeano npu 1—3% or acuara, npe6oxeaybann oT upesna mHdexwus (Shigella, Salmonella, Yersinia, Campylo- bacter). Ernoaorna. PA ce pasnita cuien mdpex- uns ¢ B-XeMOAMTHYCH CTpenTOKOK OT Ipy- na A (THIMYCH MpHMep ¢ aprpHTET pH. pesMatHunata Gorect), cAea 4peBH MH «exit (8 0-rope) Ht cate yporeHHTa- Ha uHgeKuna c Chlamydia trachomatis. Tarorene3a. [pearonaraemn natorene- THYHM MeXaHH3MH ca: 1, HMYHOKOMIICK- ceH MexaHH3eM, 2. KPbcTOcaHa peaKuutst HOpaiHt CXOMCTBO B AHTHTeHHATa CTPyKTY- pa Ha nndexumosHitTe npuMATHHTesTH H 4O- BeLUKHs TKaHeH aHTuren HLA B27, 3. mo- auuunpane Ha TBKAHHMA anTHreH HLA B 27 B aproanTuren oT enHToniTe Ha MH- qbexumosnure anturenn, Mpeapasnonarautt renerm4eH cbakTOp ¢ HOCHTeACTBOTO Ha HLA B27 anturen, orxpupaut ce npit 60— ‘0% oF naunentute ¢ PA cpeuty 8—9% HOCHTeAM Ha AHTHTeHAa B OOuLaTa Monysa- una. Kanuuana xaprama, PA ¢ 3a6onsvane, TpoABABAMLO Ce CbC 3acATAHe Ha CTABHHA alapar M HAMM Ha HIBBHCTABHH Mposi-3aGoasBarius Ha cheguiumeanama mokan / 405 BH. 3a60amBaHCTO Ce pa3BMBa OOMKHOBe- Ho 2—4 ceammunt crea npeKapana pecnit- paTopHa, upeBia WIM yporenTanHa WH- dbexuns. 3artouna ocrpo ¢ de6puanter Ht ACHMETPHYCH MOHO- HIM O/MFOApTpHT Ht no-patko ¢ NoMapTpHT. Bacarar ce npe- AMMHO CTaBHTe Ha AONHHTe KpaitHHMUM (KO- ICHHM, HeseHHH, TaIOGeApeHH, TapsaTHit, MHTepdananreainn), 1O-psAKO Ha POpHHTe KpaitHHun (rpHBHeHM, paMeHHH, AaKeTHit, IPbAHOKMONCUHH, HHTepdaranreamnn), cakpoHMHT (o6eKHOBeHO eaHocTpanen), AaKTHAMT (apTpHT Ha MpOKCHMAHH H JMC~ TAIHM MHTepalanreaiHH cTaBH HM TCHO- CHHOBHT Ha Chula npecr) Hap. He paa- Ko ce Ha6modanat GypcHTH, TeHOCHHOBH- TH, eHTesonaTHA Ocben aprput YecTo B KAMHHKaTa ce Ha6odaBatT HM KOKHO-AMTaBHYHH Mopaxke- HA — KOMIOHKTHBHT, KepaTHT, CTOMaTHT, anaHHt, Makysonary703en o6pus, erythe- ma nodosum, keratoderma blennorrhagi- cum (THIMYeH OGpHB NpH cHHApOMa Ha Reiter) u ap. Te Morat aa mpeauectsyBatT WIM a CbIreTCTByBar aprpHtta.OTHocHTen- HO paako ce Na6OAaBaT OpraHHH mopa- 2XXCHMA KATO OCTHP MPHAOUMKINT, KapAHT, Hepat. Oco6ena ibopma na PA © cunapomer na Reiter, npoawanant ce c TpHaaata: apr- DHT, KOMIONKTHBHT H ypeTpHT (BIDK CTOH- amaoaptponarnn). JlaGoparopun wacaexsanua, Yoraionst- Bat Ce AAHHM 3a BH3TaAHTeEACH Npoucc — yseanuenn CYE, CRP, no-patko 1¢BKO- unT03a.BaxkHO 3a AMarHo3ata ¢ 10Ka3Ba- HeTO Ha HHdeKUMOSHHA NpHUMHHTeET — W3CHeABAHe Ha rbpeH ceKpeT H AHTHCT- penromM3nnos THTEp (AST) np MHdbeK- Mat C B-XEMOAMTHYCH CTPENTOKOK OT TPY- na A, KonpokyaTypH W CepomOrH4Ho H3- cileaBane 3a Thpcene Ha aHTHTeAa cpelly Shigella, Salmonella, Yersinia, Campylo- bacter # Chlamydia trachomatis, 1enKo- UMTYpHA — NPM ypeTpHT, AOKa3BaHe Ha XlaMMAMMTe B ypeTpatHa HamasKa. IgM~- pesMaTonANMaAT chaxtop (TecTbT Ha Waaler-Rose) 4 aHTHHykjieapHute aHTH- teaa (ANA) ca HeraTHBnit. HoctecTBo Ha HLA B27 anuren ce ycranonssa npn 60- 90% or naunentHTe Amarnosa. Usnoasysar ce anarnocri- MHTe KpHTCpHH Ha dpeHcKata peBMato- jormuna acounauna( 1983): 1. Aprpur — acumerpuucn onnroapr- pir, Gorka B cakpoHTHaNHara o6s1acr, 2aK- THAMT, CHTeIONATHS; Entepoxoanr; Hernocn KomionKTHBHT, Yperpur wim weppHiutT 5. THIMH KOAKHO-ANTABHNHH Mpost- but (balanitis circinata, keratoderma blen- norrhagicum); 6. Pammanocr (HocHTecTBO Ha HL B27 anturen, cpamntHa anamucsa); 7, Jloxaspane Ha npHynnntess JImarnosara ce nocrasa npu Hamtne va 4 1 nonede oF Noco“eHHTe KpHTepHH Jimepenunaana anarno3a. Haii-vecto ce npasn c IOXA, aprpitH mpi Bupycuit MndeKuUMH, CeNTHYHH ApTPHTH, TpaHsit- Tope KoKc#HT, nypnypara Ha Schonlein — Henoch, LED u ap.(sixx Crasen cHHi- pom ripit pyri 3a6ossaHits), ‘SHevenue. [pose xia ce ¢ HecTepomt- HH NporHBoBEsnaTesH cpeAcTBa — ac- nupitn.(70-90 mg/kg/24h), ankaorpenak (Feloran, Voltaren)(2—3mg/kg/24h), ~ aomeraunn (2mg/kg/24h) 1 ap. Epi Mo- HO-O:MTOAPTPHT C MPOMWPAHTEAHOCT NO- neve oF 6 CCAMMULM — BBTPECTaBHO TIpH~ HOKCHHE Ha AeNO-KOPTHKOCTE POH ripenapari.IIpu TekKO Opranno Topaxe- He (OCTbp MPHALOUMKAT, KapsitT) — T0- KoxopTuKocteponan no Img/kg/24h.An- THOMOTHYHO JeYeHHe Ce Halara B C/YHaHTe Ha akTHBHa MHCKUMA WH MIpH HHCpeRIUT c Chlamydia trachomat Tipornosa. 3aGoaspanero ro npiniutt ec Grlaronpusren 13x01. BesMowKHH ca Pe~ MANBH Ha aprpHTa, KakTO H MpoTpax- paHo MpoTHuaHe C MpOMBPKHTEAHOCT MO ee oT 3 Mecetta. MHOro pAAkO ApTPHTET Moxe a epomionpa B LOXA — onnroapT- pirra dbopMa IT THM H yoReniLTeH aHKH- O3Mpalll CHOHAMIOAPTPHT. aoe Bun406 / VueGnuk no nequampus 11.2. CnaonaHnoaprponaTHH Cnomamaoaprponarumte (CIA) ca pes- MATHYHH 3aG6011BAHNA, KOTO Ce XapakTe- PH3HpaT Che 3acHrane Ha aKCHATHHA CKe- let (CAKPOHAMAYHM CTaBH, CTABHTe H BpB3- KitTe Ha rphOnaunna crBA6) H epHibepen aptput. pit tax He ce orkpHBat peBMato- inn cbaktopn (Por scwukst Kracose, kakTO H Jlpyr abToanTHtesa. Yecro ce pas- BHBAT Ha (bona Ha onpeaeren reneTHYcH IepeH, HACTHUHO AcTePMAHMpaH OT HOCH- reactboro Ha HLA B-27 anturen 1 KMaT cwomounst pasamuna or Taam Ha IOXA HaGmo.ana ce 206pe H3pazena dbauunocr. pynata Ha cnonanaoaptponatunte BKINOUBA IOBCHILIHMS AHKMUOSMpalll CHOH- awioaprput (IOAC), cnonmttioaprpnra NIpH WBCHHAHHA NCOpMaTH4YeH apTpuT (IOTcA), aprponarnsra np BesnamTen- Hite 4peBHt 3a60a1BaHHA (BU3) H cHH- ipoma Ha Paitrep (CP). Ernoaormata HM, ¢ H3KO4eHHe Ha CP ¢ HenosHata, HO MO aHanorHsa ¢ Hero ce lupHeMa, Ye OTKMONBAMLa PosA HrpastT pas- LM HHeKUMOSHH MpHAMAHTeAM, To- Ba CXBAMLAHE Ce MOAKPCTIA MHAMPeKTHO OT mo-4ecToTo passuTHe Ha CITA npu acua c AePHUNT Ha ceKpeTopeH IgA, KakTO H pH HWAKOH “peBHH 3aG01ABAHKA (MeTMAKHA, yalepoxemoparnuen Kot, GonecT Ha Crohn), np KonTO e Hapyuiena Gapnep- Hara bYHKUM Ha UpeBnata sMraBnua He YBCAMYCHA NpOHMUaeMocTTa M 3a AMrec- TaGa. 11.3 Mpunoxpueawu ce Geaesu na CIA THBHH aHTHTeHH, BK, H OT GakTepHateH npow3xoa. B natorenesata Ha Ta3H rpyna 3A60.1ABAHHA MEHTPATHO MsACTO Ce OTIC na HLA B-27anturena Hu Kppcrocano pe- aripailnte c Hero aHTHreHH — B-7, B- 12, B-13, B-40, B-42. Bsanmoaciictsncto Mexkly MUKpoopranH3Mma # B-27 anture- Ha ce OGsCHABa C TeOpHATa 3a KpBCTOCA Hata aHTHTeHHOCT, Ta ce OCHOBABA Ha OT- KpHTHTe OGUM enHTONH MexIy HLA B- 27 auturena w anTurenMte Ha upesHitte aktepun. Cropes apyra Teopitst — Ha Mo- anMunpanns aHTuren, 4pesHue Gakre- PHM MocpeacTBOM eAHH OOUL TIa3MiLA TIpo- Mens crpyktypata Ha HLA B-27 anritre- Ha, KATO MO TO3H HaYMH TO MpeBpbulaT B aBroanTuren. Kawuwunata Kapruna Ha Ta3it rpyna 3a- GonsBannsa ¢ pasHoo6pasna, Kato cbueTa HHCTO Ha ABA H MOBeYC MPH3HAKA MOXe sa ce Ha6jH0MaBa KaKTO B ONpescsICH NaLlncHT, Taka WY pagIMYHH HHAMBIUE OT cua Ht cputa cbammmis, Ha ta6.1. 11.3 ca nocouenit OCHOBHHTe KIMHHKO-1a60paTOpHH Xapak- TepHCTHKH Ha CHowWULIapTponarHntTe 11.2.1.Ceponerarupen, enresona- THA, apTponaTHa cunipom (CEA CHHJIpOM) CEA cuHapoMbt ¢ camMocTosTeina, Enresir Axcnaren Tepucbeper aprpitr— aprpir OAC +++ 0 4+ ttt ttt HOllcA + ++ tet + BY3 + ++ +t + cP ++ + tet ++ B27 (+) ANA(+) P@(+) Hpur Koxa JInrasmun CH = oles S = ++ —- + ++ = - ae mia abs ob + tet - gt one + +H TH — cunrouat or racrpo-sirrectunamnns tpaKt 5 H+, 25-50%; +44, 50-75%; +444, > 75% +, < 25!THIIM4Ha 3a ACTCKATA Bb3PacT HO3OTOTHY- Ha cannuua. [pw Hero Goannte HMaT Ha- kon of Genesute Ha CIA, Ho ca 6e3 3acs- rae Ha aKCHaIHA CKeAeT, KOCTO He 1103- BoABa Ma Ce NocTaBH AMarHo3a IOAC. Oc- HOBHHTe AMAHOCTHYHH KPHTepHit Ca: Ha- 4al0 Ha MYCKYHO-ckeleTHaTa CHMTITOMAa- THka npeaw 17 rou. BbIpacT; OTCBCTBHE Ha P@ # ANA; enttesonariia; aprpaarint WH aprput. Ceponeratipocrra ce onpeacas or autnicata Ha P® it ANA y Goamute. Ex- Tesonatustta ce Manuipectipa c GomkH Ha sULaBHNtTe MecTa Ha CyXOXKMMNINTA HsIMTa- MeHTHTe OGHKHOBEHO OKOTO KOAAHOTO Ht nerite. Crabnustt cHHapoM ce mpeactans kaTo OMToapTpHT Ha AOAMINTe KpAitHHULH HLA B-27 anturen ce oTkpitpa 6 70% oF naunenture. B noopmHata oT cayvaitTe ce paspupa CTIA 11.2.2. lOsenmaen anknso3npant CHOHAMOapTpPHT JOAC € xponmuen Bearianitteaen apt- PHT, sacaraut nepacbepHis H aKcHaTHitA CKeueT, HeCTO NpHupyKawan OT cHTesONa- THs, CeponeratHpen no oTHOMIEHHe Ha PD ANA. Yecrorara my ¢ 30/100 000 acr- cko Hacerenne, JloGpe H3paseHo ¢ npeBa- IMPAHeTO Ha MBKKHA TOM, KATO ChOTHO- 1IeHHeTO MoMMeTa:Mommuera ¢ 7:1, 3arlo4- ba Hait-uecto caea 10 roa. Bb3pact. Pa- Mitta o6pemeneHoct ce HaGsmosaBa B 65% or cayuanre, a HocTexctBo Ha HLA B- 27 anturen B 90% Kanuwana Kaprana. 3a pasitka or BbapacTHHTe 3acaraneTo Ha akcHaNHHt ckeneT ce HaGmonaBa camo B 25% B Ha- Naoto Ha sa6onspaneTo. IOAC no npa- BHO sarlouBa C HepHcbepen acHMeTpH- eH OAMTOapTpHT Ha AONHMTE KpaitHH- un. XapaxTepvo 3a Hero €, e Toit “106- pokayecTBeH”, T.¢. KbCHO HACTBITBAT Cpo- 3MBHM MIpOMeHH HW MHOFO 1OGpe ce BAM- ae or sewenne c HCIIBC, Koero ro or- amuapa ot IOXA, 3acsranero Ha aKcH- QIMMA CKeNeT Ce NposBABA C HaNMUHe Ha 3aGoanBanus Ha cheguiumernama mokan / 407 cakponaunr (chonTanna, npw HatHcK 1 amerpaxunst Gorka B CH crasi) 1/in Gorka, cKOBaHOcT H OrpanmucHne Ha ABMACHHATA Ha TPLONAMHHs crb:I6. Pa- HO Ce NOSHTHBHpa TecTbT Ha Schober, c KOTO ce H3CIeABA NOABIAHOCTTA 1 AYMOAMHHA CerMeHT Ha rpbonaunits cra, 3aryOara Ha HopMaanara ayMOa- Ha JOpaosa H TopakaaHa Kitco3a ca 110- KecHHt mpossn Ha OAC Or ekerpaaprukysapHute npospi nait-vecra (60%) ¢ enmezonamuama. 10- Ba HOHATHE BKMONBA BHINATNTEIHO-e- reHepaTHBHit NpoMeHH B OGAacTTA Ha 3a- aaBHIITe MecTa Ha JMraMeHTHTe, CyXo- AWAMSTA, cacuMMTe MAM KancyaHTe 4a koctta. KanHi4Ho ce mpeactans c Go.1- Ka 1/H1M OTOK Ha ChOTBCTHOTO 34a1aBHO aacto. OcTbp HpHAOUNKANT ce HaGsWO- aapa B 10% or saGoseniTe. 3HauTeHO 1lO-pAAKO Ce YeTAHOBABAT AOPTHT, Hapy- eH BCopACHHATA MpoBOANMOCT, Cau- da equina cHnapom, IgA nedpar, ax slonao3a JlaGoparopmure wacaeawanna ca Hectic unui Jletennero ce mpo-Bexuta OCHOBHO C HCTIBC, a or Gannoaeitcrsauntre Sulfa- salazin, He ce npenoppusar satin com, D-penicillamin Esomounstra u npornosara Ha OAC ca no-206put or Test Ha IOXA. 11.2.3. IOspennen ncopnarusen aprpuT JOMcA © xponmucn wpsnamnTereH aprpurr, BeannkHar mpestt 16 rod. Bb3- pact, npemuectnan, mpiapykasay Wn hocaeasan oT KOXKEH tcopHasie, Cpenia ce BbB ResKa BHIPACT, KATO OTHOUICHIC- To Momiteta:momueta € 2:1, HaGmona- nat ce pasamuntn antirenst ot HLA cne~ temara (B13, B17, Cw6, B27, B38, DR7), KONTO ca CBEP3AHH ¢ pasmIHM ops Ha 3a601spaneTo, XapakTepHo 3a CTAB- paKe Ht CHIVIPOM ¢ ACHMETPHAHOTO TO]408 / Vae6nuk no nequampus Hine Ha MHTepdbasanreaanute HW MeTakap- Ho-*ananreannite craBn, OGM OTOK Ha npbera — KaTo “KpeMBHpul”, 3acsra- He Ha AMCTaMMMTe MHTepdananreanHn Capit, MyTHLIMpall apTpHT, 3acarane Ha IpLOHaunns crBA6 Ges cakpomanuT. J ArHOCTHMHM KPHTepHH ca apTpHT © TH- HMMM NCOPHATHMHK 1e3HH KIM ApTpHT © Tpit oF caeANHTe NeTHpH MaIKH KpH- Tepitsl: AAKTHANT, TOUKOBHUINM AempecHnt Ha HOKTHTe (HOKTH KaTO “HalmpbCTHHK™), HicopHasonoxoGer Gps, cbamnana o6- pemenenocr ¢ ncoprasne. Jlevenneto ¢ kato mpit IOXA, Ho He ce H3non3Ba Resochin nopaiit Baouanane Ha KoAHIA cumipoM, Exonounsita i mporHosara ca Henpeackasyemn 11.2.4. Cunapom na Reiter Cunaporror na Reiter ¢ peakrisen ap- IPT, T.C. aPIPHT, OTKOYEH OF HHMeK- UMOSHH ArCHTH, KOHTO He Ce OTKPHBAT B sacernarire crap. Xapaxtepuara TpHata Ha 3a6oaABaneTO ce MpeacTaBA ¢_ApTpUT, KOHIONKTHBHT Ht ypeTpHT: Yecrorara na CP g aetckata BB3pacr ¢ HenspecTHa, Bb3HHKBa Haii-4ecTO crea undexuntst coe Shigella, Yersinia, Salmo- nella # no-panko Chlamydia. Paspnpa ce Ha renerHyer Tepe AeTepMMNMpaH oT HLA B27 (90%). 3anouna octpo 2—3 cea- MHL Cue AMApHst ¢ TepHdpepeH acHMeT- pHYeH ONMroaprpHT Ha AOsMMTe KpaiiLt- un. Tpoxkana 1—3 Meceua, 0 © Bb3- MOAHO H TpoTpaxHpano MpoTHyane, KaK- TO H PeLMANBH Ha aprpHta. CnoHanoapt- ponatua ce passna B 20-25%. Koxno- IMTABMYHUST CHHAPOM Ce MPeACTABA HOT keratoderma blennorrhagicum, balanitis cir- cinata, UepBHuMT, BarHHHT, erythema no- dosum, Makyo-nycTy103HM epymut ro JulaninTe M CTbManata, ynuepaunnt Ha ycT- Hata JMrapiua. Jlevenneto ce nposexaa c HCIIBC. PKC, antnOnormun — npw nyxaa (wia- muans) Mpornosata e 206pa. 11.2.5. Be3snannrernn apesun 3a- Ooaspanna Aprponarusra np BY3 ce xedinpa Kato HeMHdbeKUMO3CH apTpHT, NpHApyx*a- aut Gonectra Ha Crohn win YXK. Cpeuta ce mpi 10—20% H noseue or acuata c BY3 ima sa Tuna aprput — nosaprpit, Koit- TO € HECPOIHBEH, € EBOMOLUIS KATO Ha OC- HOBHOTO 3aG0NMBAHE MH OAMTOApTpPHT cbc CHOHAMANT, KOitTO ce pasBHBa pn HLA B27 (+) M4 BeaHbx BR3HUKHAT HMa Mpor- PecHpallla CBOMOLNA, He3aBUCHMa OT Ta au Ha BY3. Haii-4ectw racTpoMHTecTHHaTHH CHCTEMHH MpoABH Ca: AMAPHA, XeMaTOXe~ 3Hst, KopeMHa Gouika, 3ary6a Ha Tero, TeM- HepatypH, Hospbulane, nepHanarnn Mpo- MeHH, erythema nodosum, opannn Ae3uH, ysent, pyoderma gangrenosum. Jlewenneto ce npopexia c HCTIBC, Sulfasalazin. Tlpornosara ¢ Moro z06pa np rep qbepen aptput, Ho ce NoMpaviasa Tipu BTO- para (bopaa nopaan tpaiinutTe nopaxkerits Ha aKcMaaHHA ckeseT. 11.3. Pesmaru4una OonectT Pesmarnunara Gonect (Pb) ¢ cucreMHo 3a6OAABAHE Ha CbEAMHMTENHATA ThKAH, KoeTo 3acsira cbpueTo, crasute, LIHC, Ko- 2KaTa HW MOMKOKHATA ThKAH. Ts € HerHoit- HO YCAOAKHCHME Ha MAPEKUMH Ha FOPHHTE AMXATeNHM METHIA, MpeANaBHKAHH oF B- XeMOAMTHYeH CTpenTOKOK rpynia A. By3- Husa Haii-4ecto 2—3 CeAMMLM ce Hes MC Ab/XKM Ha CBPbX4YBCTBUTCAHOCT KbM CTPenTOKOKOBHTe aHTHTCHH.= Ennaemmosorna. PB sacra canakso it apara noaa. T»psuar npucren e Mexay 5 1 15 roa. spspact. Yecrorara it ¢ a0cta NO-HHCKa B CpaBHEHHe ¢ MHHATOTO it B. pasBuTHTe cTpann e€ 5/100 000, a camo 3a AeTeKOTO HaceacHue 1/100 000. Ermosorna narorenesa. OOutonpie- Ta ¢ ponsta Ha B-xeMoaMTHMHHA Crpertto- kok rpyna A B cTHOnOrHATa Ha PB. Hait- “ecto TOBa Ca CTpentoKoKH M TuroBe |, 3, 5, 6, 18, Kato 3aqbKUTCAHO yorOBHe ¢ NepcueTupaneTo Ha MNdeKuMsTA Hait- maiko 10 AHH. TouHMaT MexaHH3bM, no KONTO MpedlectBaMata crpentoKOKOBa MHCKUNA BOA 10 MHOXeCTBEHHTe sAe- 34H TIpH PB e Henspecten, HO UcHTpaHO MACTO 3deMa CBPBXUYBCTBHTeAHOCTTA Ha MakpoopraHi3Ma, KOitTO H3paGorBa cpe- Uy CTpentTOKOKOBHTe aHTHreHH aHTHTEMa, KPbCTOCaHO pearupaln CLC CoGCTBEHHTE ThKAaHH (MOACKYAKpHa MUMUKpHA). B noa- kpena Ha Ta3H TeopuA ca crenHMTe (baK- TH: autica Ha PB p panna spspact (1.¢. 1e- OOXOAMMOCT OT NOBTOPCH KOHTaKT); camo. 3% oT AcuaTa Ca CKIONHM Aa pasBnaT PB CAC CTPenTOKOKOB chapHHrHT; BHCOKA KOHKOpAanTHocT (20%) mp canosiunn OAM3HALIM; CKCHECHBEH AaHTHTCICH OTTO- BOP CIIpAMO CrpenToKOKOBHTe aHTHTeHH Ha 3a6oncante or PB & cpasnenue c octa- HaaMTe; acouMAMA Ha PB c BHCOKO HKBO Na excnpecha Ha B KeTRMHMA aloaHTH- ren D8/17 (ren na peamati3Ma?). Kanuwana kaprina, Outre poste ca remnepatypa (38,5°—40°C), Geaocr, oT- NaaHalocT, KOpeMHM GoKH, KOHTO 4UcCTO HpeaulectBat aprpitra. Apmpumom ce cpeuia B 75% oT GonHH- re._Toit © npeamMuo noxmaprput ¢ Mur- DUpalll xapakTep H 3aCATa MpHO roeMH- Te cian Ha aonMTe Kpaitnuun. 3aro4Ba OCTPO, ChE CHiHa GOKA 10 HeBL3MOxHOCT 3a ABWKCHHE, OcOGeHO B MbpBHTe 24h. TlpomprxnTennocrta My B e”Ha craBa ¢ or Aum 20 1-2 ceammun. Muoro 206pe ce NOWANABA OF ¢é arn. Mpesumana 6e3 ocrarbunn npomenn. Kapdum ce ycranonana 8 50%. Toit ¢ 3a6oaabarus na cbegunumeatama mokan / 409 Hait-TexKaTa nposwa Ha PB. [Mo-Hect ¢ npit MankH aeua. Exdoxapdumem niait-aecto wo- AM 20 MHYpatia Micy>uunenuns ¢ n0- KBCHO ocpopmsiHe Ha CreHo3a. Curnitit KaHTCH 3a MHTpaIHa HHcyd>HUNCHUNA ¢ BLPXOBUSIT, MOUTH XONOCHCTOAEH MyM, ¢ AYXalll XapakTep, OOMKHOBeHO Nporarupaut KbM aKcHaTa, ¢ pawINucH B Ha4aioTo Hit TeHIHTET, HO Hait-Mako 2/6 creneH c yeu aeH HI Ton, Bropit no yectora ¢ Me3omnt- acrounust iyM Ha Carey-Coombs, KoitTo © pesysTaT Ha pewaTHBHa MuTpatHa cre- Hosa. Tpit aoprna micydpHunenuns Ha 0 HopaTa ce ycTaHoBABA paHeH AMacroseH YM, KOHTO ¢ BHCOKOdpeKBeHTeH, seK- PECUCHO, C ACHMPAaTHBEH Xapakrep It ce, MyBa Haii-206pe No AcBHA CrepHaacH pro. Muokapdumom ce npeacransa © Taxit-Kap- JA B HOKOHL, KapAMOMeraIHA, rare pu TM, Nponosnst Hapyuenna (A-V 610K), sactoiina cppacuna Henocrarbanocr. II puxapdumam ce xapaktepu3uipa ¢ mpeKop- ananin Gonku 1 TepHkapano tpHene. Hn- Kora He Ce pa3BHBa caMocrosTenHo, 6e3 eHOMMOKApANT Xopea munop — 10—15%. Ava stn 10- 4ecTo MIpH MOMMYeTa, ¢ MHKOBA H3sIBA Ha 8 roa. Bpspacr. 3anouBa c eMoUMOHAIHa AAGWANOCT, NOCICABAHA OT XHMOTOHHIHO- XHICPKHHCTHYCH CHHAPOM, MpescraBAuL ce OT HeBONEBH ABHACHHS Ha MyCkyIaTy pata Ha AMUcTO, e3HKa H kpaitumuntre, KONTO Ce 3aCHABAT MIpH eMoUNMONaseH crpec M NpeMHHaBaT NO BpeMe Ha CbH Yecro ¢ w30nMpana nposBa Ha PB. [po- WKbIDKABA OT HIKOKO CCAMHUM 10 2—3 Me~ cela, Moxe Ja peuMAMBHpa. Epumema anyaape — non 5%. Bup3o- Apexonten, HecepGAU NPLETEHOBIIICH CK- 3aHTeM M0 THTOTO MH KpaiHMLUTe. Modkowcnu eo3au — 1%. HeGonesnenit NOABIKHH BLATH C AHaMeTbp Noa | CM, paznolOXeHH Hal KOCTHHTE M3pacrbult, CYXOAMINATA, CTABHUTE KarIcyH, HeCTO Ce ACOUMMpar ¢ TEKDK KapAHT. JlaGopatopan H3creaBanna. Yeennue- un CYE nt CRP, s1eka HopMoxpomna ane- Mua, HesaMDDKHTEMHA AeBKOUNTOIA OC3410 / Vae6nuk no nequampus ONCBABAHC. ZANBLAUTCHO € MOKAIBAHCTO Ha MIpeauucc BAU CTperTOKOKOBH HHMpeK- LUI Npes NOcHBKH Ha PbpAeH ceKper, on- peleAAHe Ha ANTHCTPENTOAMIHHOBHA TH- Tpp (Tovo-xKHTeTeH B 80%) Ha ApyrH aH- THCTPeNTOKOKOBH aHTHTea — aNTHCTpen- TOKHHa3a, ANTHXHAypoHHAasa, aHTHe- sokcupHOonyKteasa B (4ysersuTenHocr 95%). EKD — yanaxen P-R mtepsaa, a lip MepHkapanT — HucbK BOAT@K, THI Hit pomenn B ST cermenta. Jwarnosa. snon3gat-¢e anarnocrn4- Hutte KpHTepin Ha Tones, Tiaenu kpumepuu: Kapawr, noanaprput, Xopea, CpHTeMa anysape, NOAKOKHM BL3- au Monsanumeanu: tesmnepatypa, aprpaa- Mal, alanitesa 3a NpesMecTBaUL NpHCTEN na Pb, yawrxen PR witepsa, yBeanye- nt CYE n CRP. Jwarnosara PB ce nocraps npit 2 rap- Hit KPHTepHsL HUI CuI TIABeH HBA J10- MBANHTCAM Ha 3a MpemueeTBaua crpentokokona MHdeKuns (yBeTHUCH AST, no1oxKUTeeH rEpieH ceKpeT, Hac- Kopo mpekapana ckapaaTHa) Jlevenne. 3annxkNTeAHa XocHMTaIH- alls, MOCTeETeH PeKHM 3a MHHMYM 2 cCeAMMUM, AOPH Ges HaNMMHe Ha KapANT. 11.4. Craspen cHHapom Crasnusr cHmpom (ctapia 6ouKa, or- PAHMYCHH ABWKCHUS H/MJIM OTOK) ce cpe- ila B OKOIO 5% OT AeLaTa KATO OCHOBeH WWI TIpHapyxKanaut cHHpOM Ha 3aGons- BAH MH CbCTORHMA B AeTCKaTa Bb3PAcT (va6a. 11.4). Hacounauut KbM AMarHosaTa ca jaH- HitTe OT AHAMHe3aTa, KMHHYHOTO H3CIeL- Bane H aGopatopunre tecrone. OTroBo- pbr Ha 3 OCHOBHH BENpoca CAH 3a OC- HoBa Ha aMdepenumamara wMarnosa: Es- Ha HJM MHOrO cTaBH ca 3acerHaTH? Kak- BO € KMHM4HOTO ChCTOAHHe Ha AeTeTO — TexkO Wit JeKo? Hanmunte Ha deOpmn- Ter? ima 4 ochonnm satan: 1, Jnkenanpane Ha B-XeMOAHTHYHMA CTPeNTOKOK OT rpy- na Ac 10 anesex Kype menMunann ne- popano WAH MycKyaHO B 103a S0—100 E/kg/24h B 4 npuema (ananKaunn). 2 Awarorpaiina sropusia npodwiaxruKa c oraea creawaun npucrenn. Mponexaa ce 5 roaMHH, a npHt ocopaen cupacueH 110- pox # no-awiro. Msnoaspat ce: Ger3a- muti 1 200 000 E im. Ha 20 an naa 6 roa. spspact 1 600 000 E im. oa 6 roa Bb3pact. MenounaznHn no 200—400 000 E per os aHeBHo. [px aaeprisa KbM HeHH- uit — eputpan 200—400 mg 1 mr ANeBHO WA Oucentor. 3. Jevenne Ha octpitte nposnn Ha PB. Acnupin 75— 100 mg/kg/24h 3a 6—12 ceammun. Tpit kapaut — Dehydrocortison 1—2 mg/kg, 24h 3a BEIMOXHO Hail-KparkKo Bpewe. 4 Mpodptaxtika Ha GaxtepHaaen eHs0- Kapan mpi Aeua ¢ Kstamn Mopoust. Fpo- Beka Ce Hail-YecTO ¢ AMMHOTEH MULT HHL 110 CXeMa IPH XHpyprHsHtit HAH CTO- MaTonOrH4H mpoweaypHt TIpornosara a PB ce onpexesst or cre- nena Ha cbpacunoTo sacarane, FIpu mp- BH MIpueTen cMBprHoctTa € OKOIO 1%, a cea 10 roanHH KyMyaTHBHaTa cMBpTHOCT © 45%. pH ApyrH 3a001sBaHHA 11.4.1. 3acarane na eana (Han MaJIKO) CTaBH I. Texko KAMHHIHO CECTOAHHE H cheO- punter 11.4.1.1. Cenruunure aprpurn nporit aT Che CHIHO H3paseHa cTaBHa GosesHe- HocT. 3acsrat ce MpeAMMHO roneMtt CraBit — Taso6crpena, KONAHHA, Mic3eHHa, pa- Menna. Hait-vecr npH4unHHTeM B HCO- HaTanHHa nepHod ca P-cTpenvoKoK 1 Tpam-HeraTHBin HHdeKUMH, a Np TO-TO~ AeMM eta — CTA:HOKOKyC aypeyc, Xe- Motpiutyc, MHCBMOKOK Ht psuIKO TyOepKy- no3cH MUKOGakTepHit, 3a AMarHosata ¢3a6 onaBaHUs Ha CbeguHumeniama mokan / 411 TaGa. 11.4 Lupepenyuana duaenosa na cmasnua cundpom 1 Cunpsanit Mnpexuits I Pesaran | UL Byonaanteann saGOAABANHA Opro HH saG0rKnaNHA IV. Manurnenit saGoaapinis reais V. Apyru T | Cenmuven aprpt | Peawariuna 60 lenkemita Toxemien cimtonitr rect Octeommeant 1OXA pManaunn | Hepo6aactom | Xemoduanta arena | Meayaurr cat Auceunpau oc- | Kocrun tymopn | Xeworro6snona TeoxoNapitt wap mint Lncustr Aepmaromnosur | fOnomecka enw Xunepmooiacs dpintoma crane cinapost baxteparen en- | Cweceno crean- | Acenrmiia nex. | @u6posmaarna w MuTeaHo-TeKaH Ho saGoamBane Overlap syndrom Ckacpoaepaust BackyawTi, | Yaueposent kout Morbus Crohn | soKapaunr Perthes it Peaxratin aprpirnit Bupycum aprpurrit laiimcka Goacer Muxonzaama TyGepxyaona BaxHO M3CAeABANETO Ha CHHOBMaTHaTa TeuHocT — neBKounTen 6pok (>50 000 mm’), nloamnykateosa (290%), nonKCHO HHBO Ha THOKO3aTa, MikpOOHOTOrIMHO wacacapane. Tpu aeua cbc cence Morar Aa ObiaT 3acernaTH H ToBeYe CTaBH (narip, HeoHaTaeH NepHod, pH MpRicH Han BTOpHucH HMyHeH acuUNT). OcrpHstr xe- MaTorenen ocTeoMMeAHT MOE ja Owe NIpApyKCH OF cenTH4eH WAM acenTHMeH apTpHT B cEcesHa crana. OcreomuenutT B Meraduszata mporua c aprpanris B cb Mata crapa. /[Marnosata ¢ pentreHosori Ha (cumntrpaipeKa). Psoas-a6cuec npo- THMA C OcTpa Golka B Tax0GeApeHa craBa JLoGporo KannHHHO HacHeABaHe, KAKTO H penrTrenorpadckoTo HacaeABANe OCTABAT amarHosara Jleko Kanumuno cecroaume n deb- paanrer 1.4.1.2. Tpanauropen (roxcn4en) KOK- cur ce HaGsOJaba Hai-MecTo MpH ACH Ha sEapacr 3—10 ros., mo-4ecTo MomucTa. B HAKOM C/LYWaM HMa aHaMHe3sa 3a pecnMpa- TopHa wuieKuna WH TpapMa. [pornua ebe cabo napasen cbe6puaNTeT H BEITA- posa (nanp. M | ap. | 2») | | iMTeAHa akTHBHOCT. [porBAHTeAHOCTTA wy ¢ 1—2 ceasmun, paxo penmnsipa 1.4.1.3. Bupycmmre aprpirrn iporinat cbc 3aCArane Ha CANA (Hail-4eCTO KONAH- Ha) HAM MHOro cTaBH, YecTO ¢ aprpal- rit B pyri crasn, Ernonoris — py6co- na, Koxcaxnt At B, EBY, xenarur B, atc~ HoBMpye, naprosupye B19, xepmec CHMILIEKE H BApHUCAa, HaporitT itp. wt qbexui. B 1—15% cuca py6conna Bax- CHM ce HabmOAARA OUpsoNpexoxeHt crasen cHHapom. Aptpit/aptpaarusat ce ycranossna 6 10—37% mpi xeraruT B, uecTo NpHAPyACHH ¢ Makys10-Nany.1030H yam epitremen o6pin, JAmbepennuaanara amarnosa c IOXA c tpyatia. HMpoawnxn- TeMHOCTTA Ha aprpHtra MocTaBs AMArHO- nara, 11.4.1.4, Ocreona-ocreommr (GeHutrHeH KocTeH TyMop) ¢ Hait-yecT B TpoKCHMalt- nara 4acr Ha dbemypa Ht Jlawa cHuIHa Gones- HeHoct B Tas0GeapeHa crawa, NonsKora no6pe nosanavauia ce ot HCIBC. Ew- ing-sarcoma 1 ocreorenmust capKoM Ca 5 HMTHeHH KOCTHH TYMOpH NPCAMMHO Nps412 / VaeGnuk no nequampus TO-POEMH {Cla HW IOHOLUM, 4eCTO Ce Npen- crapat ¢ GonesHeHocT (HOULHa!) B Ta30- Geapena WAM KANN craBH, CBC HAH Ge3 HaKYIUBAHe IIL. JHexo Kanmmino exeroanne, Ges eopuanrer Oanroaprputnata dopma Ha IOXA IIporia ¢ ACHMeTPHMICH apTpHT B roseME crap Ham Karo MoHOaprpHT (KonAHHa crana B oko 75%); XpoHM4eH yBeHT B oko.10 20—45%; ANA (+) 8 60—85%. Tpit Hlo-roveMM MoMMeTa HocHTent Ha HLA B27 4 cHTesonaTHA, aprpHTET Moke Aa epomonpa B IOAC. Tpit acua ¢ xemod- Ist (OCOGEHO NIH ABA NPOXOAMLANTE) B Hait-ecTo TpawMupanitte crawl HacrDn- Ba KPLBONIANE, BOILeHL 10 CTABHO BY3Ma- lente H epositt Ha xpyutsita. TpapMara ¢ baxeH anamuectH4en Gener. KAMHHMHo- TO H PeHTreHONOFHMHOTO H3cHeABAHE CO- Marar 3a yeranonapane Ha dppaktypi, cy6- IyKcaLUMit, XOHIpOMAAaUMA Ha naTeaaTa Bouka 8 ras0Gcapena crapa, nonsaKora HpaaHMpauia KbM KOAHOTO, HakyUBaHe, Nlo-4ecTo Mp MoMucTa, HacoMBa KBM acenruaa wexposa (M. Perthes). [pu JOHOUIN H OCOGEHO NIpH TaKiBA ¢ Genes Ha XHMOFeHHTANHIBM TpAGBA wa ce MIC 1 3a enMHIMOANGA Ha raBara Ha Gea penara kocr. JLMceumpam ocreoxowapmr 6 kousHHaTa craBa npoTHa ¢ GomKa H/HIt orox. Juarnosara H B-TpHTe caytast e penT- renonorusn 11.4.2. 3acarane Ha MHoro cTaBH 1. Jleko Kamnuano cocroanne H e6pn- awrer Tloanaprputnara cbopma na FOXA npornya che CHMCTpHYCH aprpHT Ha TO- EMM HM MaQJIKH CT@BH, MPH MO-MaQIkH s1¢~ a, mk 1—3 roa. Bespact. B 10% aprpH- TT € ceponosutupen (IgM-P® +), c no- KBCHO Hayano (>12 ros.) Hc HOA Mor- Hosa. Mpw 10% or aeuata ¢ OXA aprpu- Thr ¢ cbe cHcTeMHH nposBH (M. Still) Tpornua c xapaktepen cbe6puanter, 06- PHB Ht aptpHt (4ecTO ANMCBaM B Hayato- To). Xponnyen aptput, Hanoso6asaut IOXA Moxe 1a ce Ha6mogaBa MpHt acua c MbpBMYeH HMYHEH AedHuMT (aramarioGy- JiMHeMHA, CenckTHBeH IgA aeduunt) Pesmatuunata Goaecr npotita c pests HO MOAMapTpHT/aprpairna ¢ MurpHpau Xapaktep H MOBINABALL Ce ApAMATHYHO or acnpuu. Mp Henoch-Schonlein pur- pura craBunte onmjaksanua (8 2/3 or cay- Yaute) ca GPIONPeXOAHH; OTOKBT B CTA- BHTe Ce ADAM Ha NepHapruKyzapen cleM, PAAKO Ha BBTPecTaBeH H31NB Mpuapyxapa ce oT nasamupyema nypny- pa.CraBHsi CHHLIpOM MpHt ocTpata AMM- ho6aacTHa AeBKeMHA Ce H351BsIBa Che CraB~ Ha Gorka, nNepwapruKynapen eaemM Ha BBTPecTaBeH H3NHB, YeCTO cDYcTAHH C KocTHa Gorka 1/M1n NatnatopHa Goues- HeHocT B MeTadu3HTe Ha ABITHTe KOCTH OGuKHoBeHo npemuecTBa XeMaTonOrH4- Hire npomenn, Tymopa, Meractasitpautit B KOCTHTe, KaTO HeBpobaactom (B 41%), paGaomuocapkom (18%), TyMop Ha Wilms (8%) W peTHHoGnactom (5%) cButo Aa- BaT KOCTHO-cTaBHH onsaKpanns. [pit BLBMAAMTeANM “peBuM 3a60raBanua (XYXK, M. Crohn) p 20—40% ce na6- suoaapa aprput. Ornaanasocr, sary6a Hi: Tero HM KPbB B M3NpaxHeHHsATA Ca Xa- paktepHa KAHHH4Ha TpHana, PeaxTusnn- ‘Te APTPHTH CIA UPeBHa UHeKUNs, BRIT cuHapom Ha Reiter mporHuat ¢ apTpuT Ha TONEMHTe CTABH Ha AOAHMTe Kpail- HMUM, CAKPOMIMHT, CHTeSOMAaTHS; HOCH- teactBo Ha HLA B27 anturen 6 50-90% Yecrit ca H3BBHCTABHHTe MpoaBH — Ko- HIOHKTHBHT, OCTRP yBeHT, ypeTpHtT, Ga- siaumt, cromatur. [py capKonao3a apt- PHTBT € CAMOCTOATEHA H3s1BA_B_OKONO 15% wan B pamxuTe Ha T. Hap. TpHasa na Léfgren — 6enoapo6Ho 3acsrane, erythema nodosum u aprpit,3a6onsBanus Ha Cbeguiumeanama mbkan / 413 11.5 Cucremen synye eputematogec (CJIE) Cucremuuar aynyc eputemarosec ¢ 10- IMCHCTEMHO, aBTOHMYHHO 3aG0s19BAHe, Xa- PakTepusupallio Cc C BEINAICHHe Ha cBe- AMHHTeIHaTa ThKaH H HMYHOKOMIUICKCeH, nackyauT, Paspitpa ce 8 pesytrat Ha rene- THYHa NpeAMCNOSHIUtA H Hapyuiena HMyH- Ha peryaauns Erwoaornata ¢ Hcnisectna B marore- esata Ha 3aG01NBaHCTO Hrpast pos HMY- HONOPHYHM HapyiicHita B XOMCOCTAIHHA KOHTPOA, 3acarauut HYKACAPHI HM WHTO- naaaMenn anturenit. DaxtopH oT oKo.- Hata cpeaa — caHYeBA CKCHOSHIMA, JC KapcTBCHH peakUMn 11M GaBHoAciicTBALIM BHPYCHH HHcPeKUMH, MoraT a yCKOpsT KIMHMUHOTO My pasBHTHe. Tpit CJIE ce OTKpHBaT THKANHO-cheuMdM4HH (THpCO- MOOyAKHH) H TEKAHHO-HecneUM>HINH anTiresta (anTHHyKcapHit, aHTHKOarysaH- THM H1p.). HocazeanmtTe HMaT cbutecTBe- Ho yuacTHe MpH OcTpa XeMOAMTHMHA aHe- MHA, TPOMOOLLMTONCHHA, ACBKONCHHA H XeMoparn4na JMaTe3a, KAKTO 1 MIpH OOpa syBaHe Ha AHTHECH-aNTHTAIO KOMILICKCH Te3 uMyHHM Komrtekcn (AK) ce orma- rat B TbkaHMTe, (bUKCHpaT KOMIIEMCHTA MW OMHMUNHpAT BLINATHTeAHA peaKuMA C HOCACABAMIO THKAHHO yapeAaane — HeEd)- put it ap. Hapyuren © KaeTRHHO-MesitHt- Pala HMYHEH OTTOBOP c ANCYHKUMS T-cynpecopunte K1eTkH. CJIE ce acounupa c HLA-B8, DW3/ DR3, DW2/DR2 anturenn, ¢ C4a nyace alen, ¢ MMYHOAeHUNTHH CBCTORHHA — ceackTHBen IgA ecpHUNT, xeTeposHroTeH, C2 aecdbuunt. Mpu 12% of nauncHtTHte ce OTKPHBaT POACTBEHHIL CBC CbILOTO 3a60- siapane. CJIE ¢ onncat npw eanositann Ormanaun. Mlaromopdoaorna. Ochopnata 1031s pw CJIE © WMyHOKOMMIcKceH BackyJINT © dHOpHHonaANa HeKposa, BLINAINTCAHH KoleThYH HHpHUTpATH HM CKsIeposa Ha KO- arena. Backyaurph sacara MasiKNTC ap- TEPAH M aprepHOAM Ha MapeHXHMHH Op- Fait, KOKA, MYKOSHIt H Cepo3ilit HOBDpX noctit. B 6LGpexa MK ce oraarar ppso- HavaHO B Me3aHtrHyMa, 11O-KbCHO — B cy- GeHAOTeAHOTO NpoctparicTHO (cpokaeH Ht auiby3Ho-npoaicpeparnBen Hepat) wit ilo enmTeaHaaHara crpana Ha roMepya- Hata Oa3zanHa MemOpana (MemOpanoscH Hepitt) Kannuxa, CJIE ¢ neobustacn non § uuuna Beapact. Hoka3Ba Hapacrnautta Yec- rota B jwHoUccTROTO, CHoTHoMeHHeTo mMomHYcTa:MoMYeTa ¢ 8:1 ¢ H3KTOUCHHC Ha mipearyepreta (3:1). 3aGo1BaneTo 3a- nouBa B AeTCKa Bb3pacT NpH 20% or Goa- unre, CIE nporia ¢ pasanuna crenen Ha TexecT — OT OCTPO, 3aBBPUIBAILO Cpa TAINO saGOAMBAHe, LO XPOHHAHO, HEsICHO npormuane ¢ peumMnpant xapakrep Haii-vecrn Hawaii cHMmtomit ca «pco- piuntrer, yMopa, aprpHt Hust aprpacirii it koxen Gps. TpH noBeeto or stenara remneparypata ¢ cyG*eOpitna H ce HOp- Mamanpa cnontanno, Koxnitte 1 antra- BHMHH MposIBH Ha 3aGoABAHETO ca HeCTHt i aKHOMNTeAHO pasHooopasHn. Menepy AOOGpasHusIT OOPHB € XApakTEPCH 3a OCT para basa, nlonaKora c dborouyBersitTesteHt (dar. 11.2). Waonupanustr sntcKomtert sy- hye ¢ HeobHuAcH 3a JeTcKara BL3pacr. JUpy- TH KOXHH OGPHBH, KONTO ce HaGmOnABAr nput CJIE, ca: epitremit Makyant Ht Gys103- HH esis, TOUKOBMAHH HeTHA BEPXY JU1a- hutre W xonuara, livedo reticularis, nypry- pa, erythema nodosum. Axjonenmsita ¢ 4ect cumrom. Maxysapuit i yatteposniit s1e3itit Morar sla Ce OTKPHSIT B YCTHATA KYXHHa, HOCa y pyanara. Aprpuret npit CJIE o6muaitito hia ripexostent 1 MTpHpaut xapaktep. AP- Tparrnit, MHatrit, cTaBHa CKOBAHOCT Ca uecra mposina Ha 3a6onsaBanero. Raynaud- dpenowent ce yeranonsina pit roubiIM Opott Or acuata 1 ¢ CBHP3AH C HMyHOKOMILICK- cen packyanT H KpHorto6ysnnenits Hec~ To_apIpHTbT ¢ cHMETpHYCH H_ GonesHen, hponoxupa ce oT CTY MH eMonMoHaTeH aypust Mur 112. Me mueto np LED. etpec. Honnceposutret (ntesput, mepn- KapaNtT Ht TlepHTOHHT) © XapakTepeH 3a 3a Bunpexn sacsranero MW Ha Gonspanero. TpuTe CbpaCHHM COEBE, Hail-YecT ¢ Ne pukapaurer. Yeranopsvanero Ha cbpited- Ha HedocTaTbYHocT, KapaHoMeramiMs i APHTMHN HaCOMBa KBM MHOKapautT. Bepy- KosHUsIT eHoKapanT Ha Libman-Sacks ¢ xapakTepHa npossa B ocTpara pasa Ha 60 nectta, TLiesput ce aMarHocruunpa np OkONO NosoBHaTa OT GonHHTe, 3ACLHO C Gasiapen MHeBMONIT 1 UICKOIAHA ate lekTasa. PeumuBHpauutte aGLOMMHATHH Gonku ca Muoro 4ecTH rip CIE y aeuata M CbuaBaT MoHsKOra cepuHOosHH sMdepeH- UMaJIHO-arHOCTHMHH 3ATPYILHEHHIA COC Tbp XHPYPrHecKH Kopem. Bb3IMOXKHH MIpH- MMMM MoraT sla ObaaT: ceposHT, BackysNT Ha Me3eHTepityMa H “peBHata cTeHa, nal kpeatur, nepironnr, XenaTomeraqusta 1 crumenomeranusra ca vectH, Tenepanuan para auMcaaeHonatHs ce yeraHoBsBa rip 1/3 oF acuiata HB HAKON cay4aH nopaxuta CbMHeHHe 3a HMOM H JTeBKeMHst Bupanyanero Ha ueHTpatnata HepaHa cuctema H 6bOpeunTe KoperMpa c NpexH BAeMOCTTa H NporHosata Ha 3aGoasBaHe ro. KAMHM4HHTe MposBH Ha synyca Ha LHC pxnousar: peusanspamto riaso60- IMe, NOHSKOFa ¢ XapakTep Ha MurpeHta, OFHHILHM WIM reHepaIHNaHpanH rbpYoBe Xopest, NCHXHYHH OTKIOHEHHS (HUHOCTO- BM NpomeHH, ricHxosa). Hirpaxpannamia- Ta xemoparist, pseudotumor cerebri, Tparic- BepsaqHata MieIONATHs, CHHAPOMBT Ha Guillain-Barre ca cepwo3Hu ycnoxHeHust Ha 3aGonsBaneTo. Ounata natonorna np CJIE Bkaw4Ba: ennckiepHT, MpHT H Bac KYIMTHH HW3MCHCHHA Ha peTHHara (“ut TORAH Tema”) Jlynycunst Hedput (JH) npucecrsa 8 pastHuHa cTenieH MPH BCHYKH AeUA cbc CJIE. Cnopea xnacnduxaunsira na C30 ce xapakTepu3upa B net Mopdorornynn tuna: | — nopma, I — Mesanreasen, III — doxanen npoanepatupen, IV — ast cby3en mponndeparusen, V — Mem6pano. 3H. KAMHMYCCKH NO-eKHTe cbopMH (THT II-II]) na JIH ce mpeactasst ¢ xematy pus, mporenuypus nox 1g/24h npn Hop- Maina Gx6peyna cynkuns. Yacr or ne uara ¢ HT tan w sewukn ¢ TV tun JH MMaT pelyunpana GvOpeuna cynKuits, HedporH4eH CHHLIPOM HI OcTpa GOpe4- Ha Hegocrarbunoct. IMpx tun V na JIH © BoueUt HepoTHMAHAT cHHAPOM. BEOpes Hata 6MONcHs ¢ ONpaBAaHa pH MoBeMe~ ro or seuata. Monumenure cepyMint an- THTea KbM LBoOiHOBepHKHa JLH K (A-ds. DNA) #1 xumoKomriementemusTa Kope- ampat c akTuBHocrra Ha JIH. Mporiosa- Ta Ha GBOpesHOTo 3aGonABaHe NpH AeHa- ra cbc CJIE ¢ no-cepo3na B cpapnenite ¢ BE3pacTHuTe. JaGopatopun w3caeasanus. Ocrpodya- soBnTe noKasateiM (CYE, CRP) ca nosi- WweHH, XeMaTOAOPH4HH OTKIOHCHHS Ce OT- Kpubat np nourn BeHuKHt Aeua cbc CITETaba. 11.5 Kpumepuu 3a C/E 3a6oasBanus Ha Chegunumeanama mbkan / 415 Nenepyaoo6pasen oGpite Aucxowzcn KoxcH o6pHB orouyseranrernocr Yauepauin B yerara 4 Hoca Heeposnacn aprpitr Hegpurr | a/ npotennypns nan 0,5 g/24h | 6/ natonorm4en ypunen ceaument 7. Enuecaronarisa a/ repyone 6/ newxosa Vawnoe 2 — aes (KCAAIOHCAOMMBNHA, XeMO.Tit- TH4Ha), JeBkoneHHA c aMMoneHus, TpomGousTonenns. Tecrét 3a anTHnyKate- apy autureaa (ANA) ¢ nosutneen npit howeue or 95% or Goannte. LE-KaerkH ce ycTaHOBABaT B HavazoTo M pit cK3auep- SaunnTe Ha 3a6018BaneTO. Anti-ds DNA aHTHTeAaTa ca BHCOKO CHleuMdHuHH 3a CIE it ce ceppsnar c akTHBHo 3a6011BaHe (nedput). Sm anrurena ¢ everanen or § Mankiw Hykteapni PHK, Gora Ha ypit AWH HW CBDp3aHn ¢ noannienrias. Anti-Sm. antHrenata ce cuntar naTorHoMOHMMH (oko10 99% creumd4nocr), Ho ce LoKa3- bar B 25S—30% or cayyanre. Ro wan Sjogren syndrome A (SS-A) anrurena ce cbcTOH OF awa NosMfenTiua, acouMMpaHH KbM PHK La anrurena ian Sjogren syndrome B (SS- B) © kommteke ot HyKreapen ipoctponpo- tenu 4 PHK. Antireaara kbm Ro/SSA 1 La/SSB ca xapaxtepuit 3a Heonaramnits Jy- nye cHmpom. Cepymuuat xemoauTicHt komiiiement (CH50) 4 HaAkKOH OT HeroBHTe komnonentH (C3, C4) ca Hamanenn npn AKTHBEH JHC, OCOGeHO NpH pasBHTHe Ha Hedpur. OrkpHpaneto Ha anTHKapanosn- MMHOBM MH AHTHKOaryraHTHM anTHTesa ce CBDP3Ba ¢ TPOMOOTHYHH HHLLLICHTH Kt KO- PeaMpa c (atNBO-NOSHTHBHMA TeCT 3a Jy- ec (VDRL). Amarnosa. JLuarno3ara CJIE © cnryp- Ha, Koraro or focoucnure 11 Kpurepua 4 8. Tacapar wan nepuxapaut 9. Lhnronenuts 10. Hosittuaa uMynoceponoriis a/ Anti-dsDNA antitrena 6/ Anti-Sm antwresa b/ LE-kaersuen dbcnomen 1/ danunpo-nositTHBent Tecr 3a ayec 11. Tloswrieit antiniykacapin anrirena ca wanmue (ra6a. 11.5) Jnepenumarma anarnosa ce nupani c IOXA, ocrep raomepystonedput, xemoutt- THM AHEM, TCBKe MHS, ateprisieHt step: MATHT, MHdeKIO3Ha MOHOHYK:ICO3A, pes arta GosecT, CMeceHa CLeANHiTeAHO- TbKAHHa Goueer, cenmMucmns Jlevenne. Kopruxocreponantre ca B oc Hopara Ha sevennero Ha CJIE # z03upon ka 1—2 mg/kg/24h ¢ noctenenno peay upalte No aaTepHMpauta cxeMa 10 Hait- nitckaTa ccbeKTHBHa Osa, HMyHocynpe: CHBHa Tepalina (HMypal, UMKIOocda- MHA) Ce Mpenopbuna mpi No-TeRKiITe bopa Ha 3a6o1ABaneto (IH, aynyc Ha LHC w ap.). Xmapoxenxiopoxsitn ¢ HH AMUMpAH IPH H3pascH KOKeH Ht craweHt CHHApoM. Fysic-repaniis c Meriuinpestit- 30.101 (110 10-30 mg/kg/24h B 3 noc.tcst0- BaTeANH ANH) Ce Mpwiara NpH TexKIE CK 3auepOauntn Ha 3aG0.11BAHICTO C KHBOTO- onacii yeroxnenust. Cranomyckyuit- AT CHHApOM ce NoBNsBa or HCIIBC. B sevennero Ha Gp6pewnara Heaocrare4- HOCT HMAaT MACTO AMAIH3aTa H OLOpeuna- ra TpanicnaanTauna. Tlpornosara ¢ rogoOpena sia MTEAHO npe3 nocieannre 30 roan — 85-90% of acuara npexHBabaT noBere oF 10 ro- aun. Hait-secrn npH4nnnt 3a CMbpTeH M3xOM ca: cercne, HedpuT, synyc Ha UHC. es416 / VaeGnuk no nequampus 11.6 Cmecena cbeqHHHTeIHO-TbKaHHa OolecT Cmecenara cheamnitreano-TbKanita 60- Aker € CHILIPOM, KOHITO ce XapakTepH3Hpa ¢ KAMHHUHHTe MposBH Ha CIE, IOXA, [epMATOMHOSHT Ht CKTEpOAePMHA HC BH Cok THTEP Ha aHTHTe:Ta KbM PHOOHYK:ICO- riporennt (RNP) Ermoaornara © neissecria. B narore- HesaTa BO.LCHLA PO Ma HMyHOKOMILICK- cHitsl BACKYANT csira no- 3aGonBaHeTo uecTo Momuuera Kamera kapruna picnouna aprpir, ckIepoxepMOnOAOOHH KOKHH NpoMeHn, Raynaud-cbenomen, beGpuatter, cppacs- nO 3acsrane (MepHKapanT), O6pHBH, Ha- nogzo6apamn CE wan aepmatomuositr, Muo3itT, eocareama anccyHKUMA, nM chaxenonatiia, xeriarocrmenomeranits, Oe- 1OAPOGHO 3aGorsBane, uTONeHItA. Hed- PHTET He ¢ HecTO YeaOAKHEHHE Ha 3aG0.18- Banco. Onuicann ca HeBpororituna cHsti- ToMaTHKa, MaporTHT. Typsonauaino CCTB ¢ spanpuemana kaTo 3aGo1BaHe ¢ NO-206pa MporHo3a B cpabnenne cbc CIE. Benpexit 3a1080.111- TeAMMST OTTOBOP KBM KOPTHKOCTepOH.AHt AaqeuHata nporHo3a Ha 3aGoaBaHeTO & HenapectHa, BramMoxkHa ¢ enOMOLA KbM CAE nt cknepoaepmns 11.7. Cunapom na Sjogren Kacae ce 3a XponittiHo aBToHMYHHO Bb3- HQIeHMe Ha CTBSHTE H CMONYCHH TCIM, © MocneaBaO HaMatcHHe Ha cekpeTop- Hust MM Karate. OcHopitte cHMMITO- MH Ha 3aGoqBAHeTO — cyXH O4MN (xe~ rophthalmia) 4 cyxa ycra (xcrostomia), T. nap. “sicca complex”, 4ecTo ce npeacra- BsIT 3aeRHO © Apyra cHCTeMHa Gonect Ha unureanara rekan (CIE, OXA) Ernoaorusta ¢ enspectia. Yeranons- MBH HHpHTTpaTHE OT THM bo- LWITH HM MOHOHYKzIeapHH KAETKH B CON MCHHTC H CMb3HH AUIC3H, KAKTO HB CeKpe- TopHiTe AIeI Ha FACTPOHHTECTHHATHHS, pecrimparopeH H yporenTaten Tpakt c HIPOrpecHBHa aTpodHs Ha CKIOKPHHHES napenxnm. Cunaposrs Ha Sjogren ce aco- unupa ¢ HLA-B8 1 -DR3 antirennt. 3aGoNABAHETO € PsUIKO B jLeTCKATA BbS- pacr. 3aesrar ce mpesiito Mommuera. Ha 4atoTo € HesCHO, eROOUMsITAa — GaBHo Bat ce Ma nporpecupaua. Kanunanata Kaptana 5x:1i0usa: oro- cho6us, napene cEpGex Ha oMNTe, 32- MBNICHO SPCHHe, YBEAMYEHHMe Ha MapoTHt HuTe KNeIH, HaMaTeHa BKycoBa “yBCTBI- TeAHOCT H OGOHSHME, ACarns, Apesras Tulae, eneTaKcHe, KapHo3HiH 3bOH, peut AMBUpalt GPOHXHT, MHeBMOHHS H XpoHHt- MeH OTHT. Toxaspat ce aproantuteaa — pepma- TOMAHH cbakTOpH, aHTHHYKACApHH aNTH- testa, Anti-SS-A (Ro) 1 Anti-SS-B (La) Almarnosata ce nocTans Bb3 OCHOBa Ha KAMHHYHATA XapakTepHeTHKa, cHalorpa- cus, cumTurpadus nce norsppxaana uipes GuoncHst Ha naGHamHa MyKo3a Jlevennero ¢ CHMNTOMAaTHYHO Hue OBIAKHABAHE HA MYKOSHHTE NOBLPXHOC- tu. Kopruxoctepoman i uyHocynpeco- pH ce npiwiarar pH cbeterBauin cHcTeM- Wt 3a609BaHHA 11. 8. Osennaen epMaTomMHo3HT 1Osennannat nepmaromnosut (IOJIM) © MYITHCHCTEMHO 3a602ABaHe, KOeTO ce xapakTepH3upa ¢ HerHoiHO BEaNATICHHe Npeaw BCHYKO Ha MpoKcHMaaHaTa Mycky- slatypa H Koxara, 3aGoasBaHeTo © cpas- HuTesIHO PAIKO B LeTeKaTA BE3pact. Fpe-3aGoasBanus Ha cbegunumennama mpkan / 417 crapnapa 3% of andy3Hite 3aGormpaHna Ha CbCAMHHMTeAHaTa ThKaH. Yectorata My e 1—4,3 cayyas Ha | sain. Hacerenne. MMo- “ecto GoucAyBaT MOMMYCTA, OTKOAKOTO momueta (1,6:1). 3acsra Bcaka BB3pacT, HO nait-4ecro Mexay 4 110 roa. H H3KMOGH- TeMHO pAAKO Noa 2 Ton. BbIpact Kaacnuxanna. Hama canna Kiacn- buxauna. Cnopea J. Cassidy sparaantes- HMTe MHOSHTH Ce ACIAT Ha: NOAMMHOSHT, ACPMATOMHO3HT, AepMaTOMHo3HT-nosH- MHO3HT C MAIHTHCHH 3a60ABaHHA, 1OBC- MICH AepMaTOMHO3HT, OCTpa pabOMHO- M34, MOAUMMHO3HT CBE Sjdgren syndrom WIM MIpHTOKpHBALIM ce CHHAPOMH, Erwoaornara ¢ Henanectua. Hama y6e- AMTCAHM 2aHHH 3a HHGbeKUHO3eH npH4n- HuTeA Ha 3a60nABaHeTO. BeposTHo KOK- caku B, pun, nukopHa BupycH, MUKCO- BMpycH, HAKOM HMyHH3auMH (py6coza, BLUK) urpast unampextia poss Kato oT- k04Balll MoMcHT. JLepMaToMHo3HTONO0- Gen cuHpoM ¢ onvcaH pH X-ceppsana- Ta aramarao6ysMHeEMHs, ACOUMMPAH C exO- BupycHa HHdeKUns Marorenesa. [Oxensuinisr aepmaromut- O3HT € HMYHOKOMILICKCHO MedMMpaHo 3a- Goaawane c oraarane nag n C3 KoMme- MCHT B CTCHHTe Ha MaIKHTe ChaOBe Ha Cke- sieTHata myckysatypa. Fpu KOJLM cputect- ByBa HMyHOreHeTHYHa npeaMcnosMuMA Jleua ¢ Towa 3a6% He Ca T1O-4CCTO HO- curean Ha HLA B8/DR3. Kamnwima kaprana. [pu no-roaama vact or senata c IOJM 6 npoapomarnns HepHon ca HamMue oTMaqHanocT, jecHa yMopsieMocr, GesaneTHTHe, 3ary6a Ha Ter- 10, cbe6puanter. Mp ocrpo Hayao Test CHMITOMH IposWLABAT AMM, LOKATO Tp XPOHH'4HO NporMane — ceAMMUN HM MC- ceun. Crenpa Myckyana caa6ocr 1 o6puB © MpomBLNTEAHOCT HAKOAKO MeceLa 10 1—2 roannn. Tocreannat KinHM4eH cTa~ ani Ha }OJLM © BpsctanopaBane ¢ ocTa- TMH MYCKYIHA XHMOTPO*HA, KOHTpak- TYPH M CBEHTYAIHO KaMUMHO3a. Koxen cumapom. 3acaraneTo Ha Koxa~ ta np IOJLM ¢ veer (85—100%) 1 paren cHMIITOM. Mpeaiiekunoniitre KOH 13- MCHCHHA Ca BRPXY ropHHTe KrenaN 1 OKOMO TAX H Ce XApakTepH3Hpat Cc aueUden epumem nox opmara Ha ounaa. Ho exe- TeH3OPHHTe MOBPXHOCTH Ha MeTaKapno- chananreanm, uutepdananreannn Ht no- PALKO KOCHHH H aKeTHH CraBit OOpHBDT € © CPHTEMHO-BHOeTOR OTeHDK, KoitTO eontoupa 20 ceaerpen (Gottron syndrom) Eana tpeta or cayyante ca c apeGHo- 1 caponeTHHCT epHTeM mpeAHMHo No OTKpHt THTe 4aCTH (TMUC, WHA, KpalHHun), Koi- To Moxke a Ghae H reHepatuanpan. Tpit NpeMsHaBaneTo My OcTaBaT NUrMeHTaut, KONTO ce 3abpxaT AbITO BpeMe, Koxiiit- Te yauepauntit ce cpeiar B 1/4 oT caysan Te 4 Ca He6aaronpHATeH AMarHocTHueH Ge. jer, OTOUMTe Ha KOKA H MOAKOAHA MacT- Ha TbKAH Ca OT H3OAMpAHH, OFHMUL TO KCHadH, MHWHHUM, WMS, NOAOeApHLM 10 FeHepasiM3npaHit, MO-4CCTO ¢ ILTbTHa KOH- CUCTCHIMA. PasBUTHCTO Ha KaIUMHO3a ¢ alpyr Xapaktepen cumnitom Ha IOJLM. Jlo- LININPAHA CB MOAKOAKHE, anloHnesposit, XOKMINA, (acu H MOHAKOTA B MyC- yauTe. Kawunnosara HMKora He ¢ IbpBHL CHMNTOM H ce MosBABa 4—6 Mecella 10 10—20 roan cued HayasoTo Ha 3a6018- paneto. Ha6smonasa ce B 1/4 10 nonoBH- Hata oT GomHHTe Aeua. OT Apyrute KOx- HO-/IMTaBMYHH MposBH Ce Cpeutar: TeneaH- THeKTASHH, KaTLWULIPHH TpOMeHHt B HOKeT- HOTO JOXC, MAIMApeH H TIAaHTApeH epH- TeM, JIMPABHYHH yIUCpAuMH, PHHEHBMT Ht ap. Myckyaten_ cunapom. Tpit IOJLM sacs- raneto Ha HanpeuHo HaGpasaenata Myc~ kysatypa ¢ paHeH M XapakTepeH MpusHak Cpemia ce 8 90—95% or caysautre. Hapa- gaa Ce B CHMeTpHYHO 3acarane Ha MpOK- Gumannata MyckyslaTypa (paMeneH HM Ta- 408 Tloac). XapakTepu3upa ce ¢ fporpe- cupamia MyckyaHa caGoct H Myckyana Gorka, oco6eHo npw namnauns. Taxipa gela He MoraT Aa MOBAMTHAT ppuecTe CH, He Morar Ja JcbpxkaT rlapata CH H3NpaBe~ Ha, 2a maka4Bat cTBION. JenaTa ¢ 101M He MOraT 1a CC H3NpaBAT CAMOCTOATEAHO418 / YueGnuk no nequampusa OT KACKHAIO NOAOKCHHE (CHMMTTOM Ha Gower). B xoaa Ha 3a601sBaHeTO ce Hab- MOMABAT MYCKYINM YILIBTHEHMA, XHMOT- podbitsl it KOHTpaktypH. Muoro psttko (5%) MOraT Ja Ce 3aCerHaT JMUCBH HM OKOTOON- uu, chapunreanin, xunodapanreannn 1 yckyat Ha HeOueTo (10%), HHTepKocTan- HM Hap. MycKysit 3acarane wa Apyrn pram m cHcTeMH: CroMauIMo-4peBen TpakT — xapakTepHsit- pa c anccbarns, acrmpauna, HaManeH Mo- THAMTET Ha XpaHonpowoza, 3aTpyAHeHMe B MOTOpHKaTa Ha 4epBara, #3BM Ha CTOMa- Xa H “eppara, 3acsraHeTo Ha cTOMaUIHO- ApeBHs TpakT ¢ JOU! NporHocTH4eH 6e- ich, AMNATeAMA CHCTeMa — sacsraneTo Ha Ipbaara MW AMNATeAHA MYcKysaTypa KAM- HHMCCKH Ce H3ABABA C AMCTIHES, OCTpa AM- Nave HEMOCTATBYHOCT, CKOHHOCT KbM HNCBMONM, UGposa; ewpue — AMdy3en. WWI OFHMLLCH MHOKApAHT, HapyuicHHe B HpOBOAMMOCT Ta; OTACAMTeAMA CHeTeMa — yapexaanero Ha GLOpeunte UMa BTopocTe- HCHHO 3HANCHHE Ce H3pasABa © MHKPO- CKONCKa XCMATYPHA KATO H3pa3 Ha TIOMe- pynr JLepMaToMMo3HT-NOAMMMO3HT C MaqHE- HeHH 3aGOAABANHA 3a PAsIHKa OT Bb3pact- Hin (20%) pw Aeuata ce cpeuta H3KMONH- TeAHO PALKO. JlaGoparopnm n3caeasanna, Marorio- MOHM4HH Ca NOBHUICHHTe MYCKYAHH CH- uM — kpeatnukunasa, GOT, LDH, aat- 20143. CKTPOMHOFpadusta NokasBa MHOSHT, OHONCHATA — MyckyAHa HeKpo3a “ Bpartatenne, Yaeamuennte CVE u CRP ca HecrieusmH4HH TecToBe 3a BR3NATHTeN- na peakuns, JIeBKOUNTHTe Ca Haii-4ecTO B rpanMuntTe Ha HOpMaTa WI JIeKO yBe- amucnu. ANA Morar aa Gbaat nonoxsTen- Hit B 30%, B yMepeHH THTpH; aHTHTeEsIa cpe- xy NonMMHosHT-1 aHTHreH (PM-1) ca 1o- AOKUTCAHK B 65%. Jmarnocrasim xpwrepun: |. Cumerpit- Ho gacarane Ha mpoKcHMaanata MycKyiia- typa, 2. Kaacnyeckw nepMatomHosnren Opus; 3. Yaeanuent Myckyann ensuMit — kpeaTHukinnasa, GOT, LDH, aniona- 3a; 4. EMP-nannn 3a muositr; 5. Myexya- Ha 6HONcHA, NOKasBalla HeKpo3a _BB3- nanenne, Tipu Haamune Ha 3. noseve KpHTepHs amarHo3ata ¢ CHrypHa. Jimepenunaana anarno3a. B chobpa- CHE BINGAT: NOCTHHEeKUMOSCH MITO3HIT NporpecuBHa MyckyaHa ANcTpodus, BpO- ACH MHOnaTHH, MHacTeHHs rpaBitc, TPH- XHHe1O3a, TOKCONIa3MO3a, CTepoHra MuonaTHa Hap, Jlevenne. Mpeanounrar ce PKC, Kon- TO B 3ABHCHMOCT OT TexXecTTA Ha 3a60.18- BaHeTo ce npHaarar no caeaNMTe HauHHHt 1. npeann3on no 2mg/ke/24h 1 Meceu c HOCACABAMLO HAMALIsBAHE TIO CXeMa 10 NO: app-kaillo seNeHMe; 2. mysc-Tepariis ¢ Me- Tnpeanuso10H 10—30 mg/kg B 3 moc seaopaTeann AHN; 3. arrepHipata Tepa- nis 3—5 mg/kg/24h no cxema. Mpitaarat ce Olle NpoTHBOManapHitNN npenapaTH; lMyHocynpecopH — MeToTpeKcar, UNKI0C~ NOpHH, UnKAOocdamHnA HM asaTHOrIpHH; MYyHorToGynHHH; TiaaMacepe3a; KUHC3H- Tepanua. Tlpornosara ¢ Gaaronpuatia B oKoI0 80% or cayyante, ¢ Hopman dynKuno- Hasten u3x0n; 1/5 O31paBABAT C OCTATbY- HH M3McHCHHS; JeTaeH 3x01 HMA B 7- 10%. 11.9. Cknepoxepmna Pasmuapat ce 2 OCHOBHH ChopMH Ht Ha- KONKO NoAopMHt Ha CKepoepMHsTa: 1. Cuctemna cknepoaepmisa (CCK) — ¢ nonbopai CREST cunapom Il. Jlokaaua cKneponepMus — ¢ non- qbopmu morphea 4 AMHeapHa Kanuuxa. CCK Ma noctenenio Hava~ AO WI0bIFa CBOMIOUNA C TepHoOAM Ha HHAK- THBHPAHe H CTH3OAH Ha CepHOSHH CHT HH MpOABH, MO-PsLIKO 3aBBPUIBAMIL ¢ Pe- MHCHSI H 110-4eCTO — ¢ pasIMMHH.OCTATh4= HM H3MCHCHHA H OPH CbC CMBPTCH H3XOIL.