Download - Reumatism Articular Acut
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REUMATISMUL ARTICULAR ACUT
Dr Elisabeta BadilaSpitalul Clinic de Urgenta
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Caz clinic 1
• 12 ani, sex feminin• Febra, dureri articulare – de 5 zile• Dureri si tumefactii genunchi
bilateral, ulterior dureri si tumefactii glezna dreapta si pumn drept (artrite migratorii)
• Cu 3 sapt anterior (perioada incubatie) – odinofagie - amigdalita (vindecata fara tratament)
• Obiectiv – artrite; cord normal
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Caz clinic 2
• 13 ani, sex feminin• Miscari dezordonate ale extrem
– de aprox. 4 zile - coree• Neaga istoric de febra, dureri
sau tumefactii articulare• Istoric de amigdalite repetate • Obiectiv – CV normal
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Caz clinic 3• 14 ani, sex masculin• Febra, dispnee – de aprox. 6 zile• Istoric de dureri articulare la nivelul
genunchilor, 1 sapt inainte de debutul simptomelor
• Istoric de amigdalita 4 sapt anterior – a luat ceva medicatie
• Obiectiv– fara semne de artrita (semnele inflamatorii remise); – CV
• suflu de regurgitare mitrala si regurgitare aortica; • turgescenta jugulara (insuficienta cardiaca dreapta)• tahipnee (IVS)
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• Definitie• Epidemiologie• Etiopatogenie• Anatomopatologie• Manifestarile clinice• Explorari paraclinice• Diagnostic pozitiv• Diagnostic diferential• Evolutie• Tratament• Profilaxie
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DefinitieDefinitie
• Reumatismul articular acut / febra reumatismala
• reactie inflamatorie nesupurativa post-streptococica de natura imunologica ce apare dupa o infectie faringo-amigdaliana cu streptococ de grup A, cu o perioada de latenta de 3 saptamani, si care afecteaza tesutul conjunctiv de la nivelul:
• cordului• articulatiilor• sistemului nervos central• tegumentelor• tesutului subcutanat
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Epidemiologie Epidemiologie
• Cea mai frecventa cauza de IC dobandita la copil si adultul tanar
• Incidenta, prevalenta – in scadere in tarile dezvoltate – SUA: 0,6 ‰
• Prevalenta inca ridicata in tarile in curs de dezvoltare– Africa : 0,3-15 ‰
• Prevalenta in Romania: 2,1 – 2,4 ‰• Boala dependenta de conditiile socio-economice
– favorizata de aglomerarea in colectivitati
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Epidemiologie Epidemiologie
• Varsta de aparitie a primului puseu: 6 -15 ani
• Foarte rar < 5 ani, nu apare > 40 ani !• Rata de recurente in primul an: 50%• Recurente in primii 5 ani ! profilaxie• Faringo-amigdalita streptococica: in 33-
58% pauci/asimptomatica ! profilaxie dificila
• RAA - la 3% dintre faringo-amigdalitele streptococice netratate
• Amigdalectomia – nu confera protectie !
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Agentul etiologicAgentul etiologic
• Streptococul β hemolitic grup A : – evidente puternice, dar indirecte
(clinice, epidemiologice, imunologice)
– serotipuri 80– actual - orice serotip poate
provoca faringita si RAA – recaderi numeroase cu serotipuri diferite !
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Agentul etiologicAgentul etiologic
– serotipuri “reumatogene” • 1, 3, 5, 6, 14, 18, 19, 27, 29 tipul M• organismul produce Ac antistreptococici care interactioneaza cu tesuturile umane datorita similaritatii antigenice
• raspuns imunologic intens la proteina M– caracteristici structurale distincte
domeniu antigenic terminal similar cu tesutul cardiac uman
• puternic incapsulati• rezistenti la fagocitoza
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Agentul etiologicAgentul etiologic– serotipuri “reumatogene”
mimetismul molecular• Cardita
– proteinele M streptococice si NABG (N acetil beta D glucoz-amina – Ag carbohidrat imuno-dominant al grupului A) epitopi comuni cu miozina miozita focala Ac celulele endoteliale umane reactioneaza cu endoteliul valvular (cu laminina din matricea proteica extracelulara) valvulita
• Coreea – NABG Ac monoclonali afecteaza
celulele neuronale printr-o proteinkinaza
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Patogenie
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PatogeniePatogenie
• raspuns imun inadecvat la inf. streptococica– persistenta infectiei faringiene– raspunsul imun anormal– ! variabilitatea interindividuala – numai unii
produc Ac titru dupa fiecare inf. strepto• predispozitie genetica
– antigen specific celulelor B – D 8/17+• la 99% pacienti cu RAA (vs 14% la control)
– incidenta crescuta a antigenelor HLA clasa II
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PatogeniePatogenie
• Imunitatea - predominant umorala:– RAA apare la 3 sapt. de la inf. streptoc– nu exista material genetic streptococic
la nivelul leziunilor cardiace reumatismale
– serotipurile reumatogene proteine antigenice Ac: endoteliu si endocard valvular (fara efect citotoxic; Ac modifica antigenicitatea unor componente proprii ale organismului secundar autoimunizare)
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PatogeniePatogenie
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PatogeniePatogenie• Antigene extracelulare ale
streptococilor – hemolizine O si S– streptokinaze– hilauronidaze– DNA-aza
• nu au rol patogen, se folosesc pentru diagnostic• cea mai utilizata - ASLO = titrul anticorpilor
antistreptolizina O
• Imunitatea celulara– rol in persistenta leziunilor granulomatoase
si agravarea leziunilor valvulare
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Anatomopatologic
• faza acuta a RAA– reactie inflamatorie exudativa si
proliferativa• la nivelul tesutului conjunctiv sau colagenului• afecteaza predominant:
– cordul – articulatiile– creierul– tesutul cutanat si subcutanat
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AnatomopatologicAnatomopatologic
• Degenerescenta fibrinoida a colagenului– modificarea structurala de baza– tes. conjunctiv interstitial – edematiat,
eozinofilic; fibrele de colagen – se fragmenteaza, se dezintegreaza
• Leziuni proliferativ - granulomatoase– infiltrate cu celule mononucleare –
celule fibro-histiocitare modificate – celule Aschoff
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Nodulii Aschoff
– patognomonici pentru cardita reumatismala
• localizari frecvente: SIV, peretii VS, urechiusa stanga
– nu se intalnesc si in alte organe afectate: articulatii, creier
– structura: necroza centrala + coroana (fibroblasti, plasmocite, limfocite, cel. gigante cu 1-7 nuclei)
– persista multi ani dupa atacul RAA, chiar la pacienti fara sindrom inflamator
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Nodul Aschoff miocardicNodul Aschoff miocardic
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AnatomopatologicAnatomopatologic
Cardita reumatismala = pancardita in faza acuta
• Leziuni miocarditice– miocardita focala cu noduli
reumatismali oligosimptomatica– miocardita difuza interstitiala
IC acuta– infiltrarea tesutului de conducere tulburari de conducere AV
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AnatomopatologicAnatomopatologic
• Leziuni endocarditice
– la nivel valvular extravazarea limfocitelor prin endoteliu leziuni exudativ-proliferative, ± verucoase, necroza fibrinoida
• versant atrial VM, versant ventricular VAo
• rar VT, cordaje, inel fibros; nu pe VP– vegetatii in “boabe de mei” –
contin fibrina, Tc
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AnatomopatologicAnatomopatologic
• inflamatia initiala insuficiente valvulare
• cronicizarea cicatrici ± calcificari– distructie progresiva aparat valvular– valva se stenozeaza prin stramtare inel,
fuziune comisuri, scurtare cordaje SM nu apartine niciodata primului episod de RAA!
• Leziuni pericardice– pericardita – la 10%– niciodata pericardita constrictiva
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Valvulita mitrala in RAAValvulita mitrala in RAA
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Valvulita aortica in RAAValvulita aortica in RAA
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Stenoza mitrala in RAAStenoza mitrala in RAA
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Manifestari clinice
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Artrita
• mai frecventa si mai severa la adolescenti• frecvent simptomul de debut• artrita febrila• caracter migrator• afecteaza articulatiile mari: genunchi,
glezne, pumni, coate• debut frecvent la membrele inferioare• tipic – 6-16 articulatii; rar – monoartrita• ameliorare rapida (24-48 h) dupa ASA –
test dg.
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Artrita
• artralgiile – frecvent mai intense decat semnele inflamatorii
• afectarea unei articulatii – max 1 sapt
• evolutia naturala frecvent intrerupta de administrarea de AINS
• radiografia – adesea fara modificari• lichidul articular – steril• complementul seric - normal
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Cardita
• Pancardita• la 50%• debut precoce – in S1 la 50%• manifestari cu grade diferite de
severitate• sufluri – nou aparute / modificarea celor
preexistente– insuficienta mitrala – frecvent– insuficienta aortica izolata – rar– stenoze – tardiv – prin cicatrici si
calcificari
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Endocardita
• suflu sistolic mitral– IM organo-functionala la 70-80%– prin edem, hipotonie pilieri, vegetatii
• suflu diastolic aortic – la 10-20%• suflu diastolic apical
organofunctional– suflul Carey Coombs umplere prea
rapida • suflurile dispar in evolutie
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Miocardita
• de la forme fruste la IC letala• tahicardie, extrasistole• galop• sufluri prin dilatari inel
valvular• cardiomegalie• fenomene de IC
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Pericardita
• fibrinoasa – durere precordiala– frecatura pericardica
• exudativa – dispare durerea – opresiune toracica +
anxietate– pana la tamponada
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Cardita • ECG
– tulburari de conducere AV– tahicardie, aritmii
• Rgf CP– cardiomegalia
• Ecocardiografia• Scintigrafia
– prezenta afectarii miocardice– nespecifica pentru RAA
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Dg + cardita
• suflu cardiac organic nou aparut
• dilatatia cardiaca• aparitia IC congestive• prezenta frecaturii pericardice
RAA “linge” articulatiile si “musca” inima!
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Radiografia toracica Radiografia toracica
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Valva mitrala in RAA - echoValva mitrala in RAA - echo
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Valva aortica in RAA-echoValva aortica in RAA-echo
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Coreea Sydenham, coreea minor“Dansul Sf. Vitus”
• la 20%
• afectare neurologica - miscari involuntare, necontrolate, aritmice, insotite de oboseala musculara si tulburari emotionale
• miscarile coreice– mai accentuate pe o parte a corpului, dar
rar apar unilateral (hemicoree); frecvent la fata, extremitati
– agravate la efort, emotii
– dispar caracteristic in somn si dupa sedative si neuroleptice
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Coreea Sydenham
• oboseala musculara– “semnul mulsului”
• tulburarile emotionale– crize de plans, agitatie– uneori psihoze tranzitorii
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Coreea Sydenham
• de 2 ori mai frecventa la sexul F• apare tardiv in cursul bolii, dupa
celelalte manifestari (dupa 3 luni; 9 luni de la infectie in medie)
• evolutie autolimitata, nu lasa sechele• risc crescut de recurenta a RAA:
necesita profilaxie!
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Nodulii subcutanati
• fermi, nedurerosi • tegumentul suprajacent – frecvent
neinflamat, neaderent la nodul• diametrul – mm 1-2 cm• localizare – suprafete osoase, in
apropierea tendoanelor, occiput, coaste• nr variabil de la 1 la 12; uzual 3-4; cand
sunt mai multi – frecvent sunt simetrici
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Nodulii subcutanati
• durata – 1 sau m.m. saptamani; rar > 1 luna
• dg.d cu nodulii reumatoizi – dureaza mai putin si sunt situati adesea chiar pe olecran (in AR – la 3-4 cm distanta)
• apar dupa prima saptamana de boala si de obicei la pacientii cu cardita
• la < 3%
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Noduli subcutanatiNoduli subcutanati
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Noduli subcutanatiNoduli subcutanati
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Eritemul marginat
• rash nepruriginos, roz sau rosu pal• localizare – trunchi, mai rar pe partea
proximala a membrelor, nu la nivelul fetei• extensia leziunii – centrifug, tegumentul
din centru revine la normal• margini continue aspect inelar eritem
inelar• caracter fugace – apare, dispare, reapare in
decurs de ore• accentuat dupa baie fierbinte
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Eritemul marginat
• frecvent apare precoce in cursul bolii• persista sau poate reaparea cand alte
manifestari ale bolii au disparut• uneori poate aparea tardiv sau chiar in
convalescenta• frecvent apare doar la pacientii cu
cardita• la < 5%
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Eritem marginatEritem marginat
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Eritem marginatEritem marginat
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Criteriile de diagnostic al RAA(Jones 1944, reactualizate AHA 1992)
Criteriile de diagnostic al RAA(Jones 1944, reactualizate AHA 1992)
Manifestari majore
Manifestari minore
CarditaPoliartritaCoreeEritem marginatNoduli subcutanati
Manifestari clinice: Febra
Artralgii
APP de RAA sau boala cardiaca reumatismalaManifestari paraclinice: Reactantilor de faza acuta (VSH, PCR) Alungirea intervalului PR pe ECG
Dovezi de infectie streptococica:Titru crescut / in crestere al Ac antistreptococici (ASLO, s.a)Izolarea streptococului grup A din culturile de secretii faringiene Test direct rapid pozitiv pentru antigen carbohidrat streptococic grup AScarlatina recenta
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• 2 criterii majore • 1 criteriu major si 2 minore, cu obligativitatea prezentei dovezilor de infectie streptococica
Diagnostic pozitiv de RAADiagnostic pozitiv de RAA
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ECG
• alungirea PR - la 30-40%• !!! BAV I nu semnifica
miocardita• modificare functionala a nodului
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ASLO
• testul cel mai frecvent utilizat• pozitiv la 80% pacienti cu RAA• variatii cu varsta, anotimpul, zona
geografica• titrul normal la copiii de 6-10 ani – 200-
300 u Todd/ml• infectia streptococica faringiana peak la
4-5 sapt (cand RAA deja evolueaza) utila determinarea titrului in dinamica
• titrul scade lent in 6 luni• titrul nu e proportional cu severitatea bolii
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Ac streptococici
• Ac anti DNA-aza B• Ac anti-hialuronidaza• anti streptozym test
– eritrocite de oaie cu Ag streptococice diferite contact cu ser bolnav aglutinare
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Probe laborator
• reactanti de faza acuta– VSH, fibrinogen, PCR– cresc in faza acuta a bolii; normale la pacientii
cu coree– VSH
• util pentru monitorizarea bolii• ! poate fi crescut in contextul anemiei (PCR
neinfluentata)
• leucocitoza – variabila• anemie inflamatorie cronica
– frecvent usoara– caracter normocrom normocitar
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Diagnostic diferential• febra prelungita
• endocardita lenta – la pacient cu factor favorizant
• osteomielita – hemoculturi pozitive
• alergia medicamentoasa • manif. cutanate, febra, artralgii
• suflurile cardiace• sufluri functionale
• valvulopatii vechi
• pericardite si miocardite de alte etiologii
• IC la un vechi valvular
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Diagnostic diferential
• manifestarile articulare• artrite piogene• boala serului • leucemii acute• purpura Henoch-Schonlein• colagenoze – LES• necroze osoase aseptice• artrita TBC• sarcoidoza
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Artrita reactiva poststreptococcica
• entitate distincta ???• artrita migratorie• perioada de latenta - 1-2
saptamani• raspuns slab la aspirina/AINS• severitate a artritei• fara cardita• manifestari extraarticulare:
tenosinovite, afectare renala
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Sechelele RAA - valvulopatiile
• frecvent la 10-20 ani dupa primul atac de RAA
• principala cauza de valvulopatii dobandite
• la ½ din pacientii cu cardita initial• valva mitrala – cel mai frecvent afectata
– calcificarile valvulare, fuziunea comisurilor stenoza mitrala
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Evolutie. PrognosticEvolutie. Prognostic• Recadere = reaparitia semnelor clinice/
paraclinice ale bolii la intreruperea tratamentului (rebound)
• Recurenta = aparitia unui nou episod de RAA dupa o noua infectie streptococica (cu alt serotip) la peste 2 luni de la intreruperea tratamentului– frecventa maxima in primul an, incidenta
crescuta in primii 5 ani
Prognostic• foarte bun la cei cu afectari extracardiace• relativ bun la cei fara sechele valvulare• rezervat la cei cu sechele valvulare
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TratamentObiective
TratamentObiective
• Eradicarea infectiei streptococice
• Tratamentul simptomatic al manifestarilor acute
• Profilaxia recurentelor
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Tratamentul curativ- antibiotic -
Tratamentul curativ- antibiotic -
• Eradicarea infectiei streptococice– indiferent de prezenta sau
absenta faringitei la momentul diagnosticului RAA
– Penicilina V p.o. 10 zile• 250 mg U x 2-3/zi la copii• 500 mg U x 2-3/zi la adulti
– Penicilina G i.m. 10 zile• 2,4-3,2 mil. U /24 ore in 4 prize
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Tratamentul curativ- antibiotic -
Tratamentul curativ- antibiotic -
– Benzatin penicilina G (Moldamin) i.m. priza unica
• 600 000 U la copii < 27 kg
• 1,2 mil U la copii > 27 kg si la adulti
– Eritromicina succinat p.o. 10 zile• la cei alergici la Penicilina • doza 40 mg/kgc/zi, in 2-4 prize• alternativa – azitromicina – 5 zile
– Cefalosporina p.o.• Cefalexin, cefaclor, cefuroxime
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Tratamentul simptomatic- antiinflamator -
Tratamentul simptomatic- antiinflamator -
– RAA fara cardita• Aspirina
– ameliorare rapida a artritei– doza 80-100 mg/kgc/zi la copii si 4-8 g/zi
la adulti– concentratia serica terapeutica 20 mg/dl– durata variabila - pana la disparitia
simptomelor sau a sindromului biologic inflamator
– scazuta treptat in 6-8 sapt.• Corticoizi
– numai in poliartrita severa fara raspuns la Aspirina
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Tratamentul simptomatic- antiinflamator -
Tratamentul simptomatic- antiinflamator -
– RAA cu cardita• Tratamentul clasic al IC• Prednison
– de la inceput– doza 1-2 mg/kg/zi 2-4 saptamani– suprapus cu Aspirina in ultimele 2
saptamani – reducerea dozei cu 5 mg la 4-5 zile si se
continua numai cu Aspirina pentru a preveni recaderea
– RAA cu coree• sedative, haloperidol, izolare
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Tratamentul igieno-dietetic
• repaus la pat – scurt– cardita – 3-12 sapt, cu
mobilizare prudenta• alimentatie completa,
echilibrata• corticoterapie
– regim hiposodat
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Tratamentul profilacticTratamentul profilactic
• Preventia primara– Benzatin penicilina (Moldamin)
• 1.2 mil u im doza unica – Penicilina V
• 250 –500 mg x 2-3/zi p.o, 10 zile
– Eritromicina • 40 mg/kg/zi in 2-4 prize, p.o, 10 zile• la cei alergici la Penicilina
– Cefalosporine gen. I si II • Cefuroxime, cefaclor, cefalexin• 10 zile
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Tratamentul profilacticTratamentul profilactic
• Preventia secundara– Benzatin penicilina (Moldamin) i.m.
• imediat dupa rezolutia episodului acut• doza 1.2 mil U/luna (? la 3 saptamani)• durata variabila
– RAA cu cardita si valvulopatie reumatismala in antecedente - minim 10 ani, pana la 40 ani sau preferabil toata viata
– RAA cu cardita fara valvulopatie - minim 10 ani sau pana la 40 ani
– RAA fara cardita - minim 5 ani sau pana la 21 ani
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Tratamentul profilacticTratamentul profilactic
• Preventia secundara– Penicilina V p.o.
• 250 mg U x 2/zi– Sulfadiazina p.o.
• 500 mg/zi la copii < 27 kg• 1000 mg/zi la copii > 27 kg si la adulti
– Eritromicina • 250 mg x 2/zi• la alergici
NU Tetraciclina: rezistenta peste 50%!Vaccin in studiu impotriva Str. grup A
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Tratamentul profilacticTratamentul profilactic
• Preventia secundara
– trebuie sa continue si dupa protezarea valvulara !
– nu inlocuieste profilaxia endocarditei bacteriene !
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MULTUMESC PENTRU ATENTIE!