vaskulitisi u djece
DESCRIPTION
medicineTRANSCRIPT
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• Vaskulitis: Upala krvnih žila koja dovodi do oštećenja krvnih žila i tkiva
• Klasifikacija vaskulitisa (Chapel Hill Consensus Conference 1994)• Vaskulitisi velikih krvnih žila(2)• Vaskulitisi srednje velikih krvnih žila(3)• Vaskulitisi malenih krvnih žila (velik broj)
• ANCA-udruženi (5)• Non-ANCA-udruženi (velik broj)
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Primarni vaskulitisiVelike krvne žile
Takayasu arteritisGiant cell (temporalni)
arteritisSrednje velike krvne žile
Poliarteritis nodosaKožni oblikSistemni oblikCogan’s syndrome
Kawasaki diseaseMalene krvne žile
Henoch-Schonlein purpuraHipersensitivni vaskulitisPrimarni angiitis CNS-a
ANCA-pozitivni vaskulitisiWegenerova granulomatozaMikroscopski periarteritisChurg–Strauss sindrom
Hunder GG, Wilking AP. Classification of the vasculitides in children.UpToDate 2005
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Sekondarni vaskulitisiVaskulitisi vezani uz infekcije
Hepatitis virusiHerpes virusi (EBV, CMV, varicella)
Vaskulitisi u bolestima vezivnog tkiva (kolagenoze)Juvenilni dermatomiositis,Sistemski lupus eritematodes,Juvenilni idiopatski artritisHipokomplementarni urtikarijalni vaskulitis
Vaskulitis izazvan lijekovimaVaskulitis u malignim bolestimaVaskulitis po transplataciji organa
Pseudovaskulitični sindromiMyxomaEndocarditisSneddon syndrome
Vaskulitis sa značajnom genetskom komponentomSindromi periodičke vrućiceBehchetova bolest
Hunder GG, Wilking AP. Classification of the vasculitides in children.UpToDate 2005
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• Giant cell arteritis (prije nazvan temporalni arteritis)
• Takayasu arteritis
• Manifestiraju se u odrasloj dobi
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• Zahvaća žene < 40 godina• Treći po učestalosti
vaskulitis u djece• Prosječna dob 11.4
godina• i u dojenačkoj dobi
• Sistemni znaci• Hipertenzija,
kardiomegalija• Klaudikacija ruku,
vrtoglavice, promjene vida
Lječenje: glukortikoidi, imunosupresivi , anti TNF
Angiografija
MR angiografija
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Polyarteritis nodosa (PAN)› Kožni› Sistemni› Koganov sindrom
Kawasaki bolest
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o akutni, samoograničavanjući vaskulitis o dojenačka i rana dječja dobo Japan, 1967., dr. Tomisaku Kawasaki o vodeći uzrok stečene bolesti srca u
dječjoj dobi (Amerika, Europa, Azija )
o Sindrom vs bolest ? (LTE; Rheumatology, 2006. Etiologija,patogeneza?)
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Incidencija: < 5 god.Japan 112:100 000SAD: Azijsko pod. 32.5:100 000
Afro-amerikanci 16.9:100 000 Latino amerikanci 11.1:100 000
Bijela rasa 9.1:100 000
Recidiv 3% (6% >6 g.)Pozitivna obiteljska anamneza 1% (pojava u
braće/sestara unutar godine dana 2,1%; 50% unutar 10 dana; blizanci 13%)
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o zimsko i rano proljetno dobao dječaci: djevojčice =1,5-1,7:1 o 76%-90%*<5 godina.
o Mortalitet (15.-45.dan): Japan 1,25% (dječaci+ kardijalne sekvele- 2,35%)
SAD 0,17% *Pediatric Clinics of North America
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o Infektivni uzročnik (klinički,epidemiološki, laboratorijski parametri): ubikvitarni uzročnik+ genetska predispozicija
o Bakterijski superantigen-toksin (Stafilokokni toksin; Leung i sur, 1993.)
o IgA, IgE (alergijska osnova)o Lijekovi, pesticidi, kemikalije, teški metali
(živa)o ARI, ovlaživači prostora, ustajale vode
??
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Kawasakijeva bolestKawasakijeva bolestGeneralizirani sistemski vaskulitisGeneralizirani sistemski vaskulitis
Jedinstvena patohistologija: u stijenci krvnih Jedinstvena patohistologija: u stijenci krvnih žila nalaz makrofaga i plazma stanica koje žila nalaz makrofaga i plazma stanica koje proizvode IgAproizvode IgA
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1. Temperatura (100%), 5 i više dana, nereagiranje na antibiotsku th.
2. Konjuktivitis (85%), negnojni, obostran3. Limfadenopatija (70%), cervikalni, akutno,negnojni,
jednostran4. Osip (80%), polimorfan, bez vezikula i krasta5. Promjene usne šupljine (90%), usnice suhe, crvene,
otečene, malinasti jezik 6. Promjene na ekstremitetima (70%), akutni eritem i
edem šaka i stopala, zatim ljuštenje koje započinje s prstima
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klinički kriteriji
Vrućica
>39 C; 5 ili >dana; antibiotici Ø
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1. Konjuktivitis (negnojni)
Bilateralan; bezbolan; bez eksudata
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2.Promjene na usnama i oralnoj sluznici:
Eritem, suhoća, ragade, krvarenje usana
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Malinast jezik
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3. Egzantem
Polimorfni; akutna faza; trup-lice, ekstremiteti, perineum
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4. Promjene na dlanovima i tabanima
Eritem
edem šaka i stopala
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4. Promjene na dlanovima i tabanima
edem šaka i stopala
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4. Promjene na dlanovima i tabanima
Deskvamacija
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5. Negnojni limfadenitis
Unilateralno,>1,5 cm anteriorni segment,tvrdi, bezbolni, negnojni
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Vrućica 5 ili> dana +4 (5) klinička znaka
Vrućica 5 ili> dana +< 4 klinička znaka uz bolest koronarnih žila (UTZ, angiografija)
Inkompletna/atipična Kawasakijeva bolest
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Kardiovaskularni sustavMiokarditis, perikarditis, valvulitis, kongestivno srčano zatajenjePromjene na koronarnim arterijama/aneurizmePromjene na ne-koronarnim arterijamaMišićno-koštani sustavArtritisArtralgiaGastrointestinalni sustavProljev/povraćanjeHepatalna disfunkcijaHidrops žučnog mjehuraCentralni živčani sustavIritabilnostAseptični meningitisSenzo-neuralni gubitak sluhaGenitourinarni sustavUretritis
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Vodeći uzrok dugoročnog morbiditeta i mortaliteta: perikard, miokard, endokard, zalisci, koronarne arterije (aneurizme 15%-25%): proksimalna LAD i proksimalna RCA;LMCA;LCX; distalna RCA
Klasifikacija: promjer (<4 mm-ektazija; >4 mm aneurizma: AHA male <5 mm;srednje 5-8 mm; velike>8 mm).
Z-score =>2,5SD (dg bez drugih klin kriterija!)
UTZ u akutnoj fazi + 6-8 tjedana od početka bolesti ili češće
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1. Aspirin: 80-100 mg/kg/dan podijeljeno u 4 doze (protuupalno); potom 3-5 mg/kg/dan (antiagregacijski) (6-8 tjedana)
Reyeov sindrom !
Preosjetljivost- dipiridamol
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2. IVIG: smanjuje kliničke manifestacije bolesti i učestalost te težinu pojave koronarnih aneurizmi2g/kg (+ perzistirajućoj/rekurentnoj vrućici 36(?) do 48 sati nakon terapije)
Rizik prijenosa infekcije-krvni derivat!Anafilaksija!Aseptički meningitis!
Shiraishi i sur., World J Pediatr 2007.:terapija IVIG u dozi od 1g/kg između 5.i 7. dana bolesti može imati isti učinak kao i standardni protokol
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3. Glukokortikoidi: primjena upitna; opravdano nakon 2-3x IVIG
“pulse” metilprednizolon 30 mg/kg iv
4. Biološka terapija
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• Non-ANCA-udruženi
• Infekcijski (Hep B, Hep C, HIV, Parvo, CMV, EBV)• Kolagenoze (RA, SLE, Bechet’s, Sjogren’s)• Lijekovima izazvani (cutaneous leukocytoclastic
angiitis)• Henoch-Schonlein Purpura (HSP) – IgA depoziti• Goodpasturova bolest• Crioglobulinemia• Upalna bolest crijeva• Urtikarijalni vaskulitis • Paraneoplastički vaskulitis
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Henoch- Shonlein purpura Henoch- Shonlein purpura Anafilaktoidna purpuraAnafilaktoidna purpura
► Najčešći vaskulitis u djeceNajčešći vaskulitis u djece ► Manje krvne žile Manje krvne žile
(koža, zglobovi, GIT, bubrezi, CNS)(koža, zglobovi, GIT, bubrezi, CNS)► Simptomatologija- KSimptomatologija- Klasilasičnačna tetrad tetrada:a:
Purpura –potkoljenice, natkoljenice (100%)Purpura –potkoljenice, natkoljenice (100%) Artritis (veliki zglobovi) (75%)Artritis (veliki zglobovi) (75%) GIT (50%)- povraćanje, kolike, hematemeza, GIT (50%)- povraćanje, kolike, hematemeza,
melenamelena Bubrezi (25%) nefritis, Bubrezi (25%) nefritis, nefrotski sy.nefrotski sy.
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90% Slučajeva u dječjoj dobi› Vršak 4-7godina
Muški spol:Ženski (1.5:1) 50% nakon virusne ili streptokokne ARI
gornjih dišnih puteva Bubrežna bolest teža u odraslih
› Rijetko progresija u renalnu insuficijenciju› IgA depositi biopsija bubrega
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Leukocitoklastični vaskulitis sa IgA depozitima
Direktna muoflorescencija kožeBiopsija kože.
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INFEKCIJE ARI Morbilio Rubella Parvovirus B19 Mycoplasma Coxsackie
virus Toxocara Amebiasis Salmonella
C.difficileC.difficile H.pyloriH.pylori AdenovirusAdenovirus LegionellaLegionella TuberculosisTuberculosis MumpsMumps StreptococcusStreptococcus grupa A grupa A BartonellaBartonella HaemophilusHaemophilus Morganella morganiiMorganella morganii
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Hrana Cjepiva Lijekovi
› Vancomycin› Streptokinase› Ranitidine› Cefuroxime› Diclofenac› Enalapril› Captopril
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Suportivno› Nesteroidni antireumatici
Prednisolone 2mg/kg/dan› Nefritis› GIT ?