Artrita Idiopatica Juvenila Nov 2009
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Transcript of Artrita Idiopatica Juvenila Nov 2009
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Artrita idiopatica juvenila (AIJ) - artrita reumatoida juvenila - artrita cronica juvenila = sinovita persistenta, cu debut sub varsta de 16 ani, sinovita ce afecteaza una sau mai multe articulatii, si evolueaza cel putin 6 saptamani; toate celelalte cauze de artrita persistenta trebuie sa fie excluse.
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ETIOPATOGENIEETIOPATOGENIE
• Factori genetici– susceptibilitate genetica
• Factori infectiosi– au fost izolate variate microrganisme in
sinoviala bolnavilor, dar nici unul nu s-a dovedit responsabil de producerea bolii
• Ipoteza imuna – Tulburǎrile imunologice includ
• producţie de autoaticorpi, • perturbarea producţiei de citokine • rǎspuns imun (la agenţii infecţioşi) modificat
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• Antigenul e prezentat celulelor T de catre macrofage, celule B, celulele dendritice, fibroblasti, celule endoteliale
• Activarea celulelor T stimuleaza productia de limfocite T si B
• Eliberarea citokinelor TNF-, IL-1 si IL-6 determina – Expansiunea policlonala a cel T– Stimularea productiei de prostaglandine, neutrofile,
complement, proteaze, enzime lizozomale – • Migrarea cel inflamatorii in lichidul si tesutul
sinovial• Cresterea permeabilitatii vasculare
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FORMA CU DEBUT SISTEMICBoala Still
• E forma cea mai rara dar adesea cu aspect dramatic
• Tabloul clinic e dominat de manifestarile extra-articulare
• Debutul - frecvent la varsta mica (uneori in primul an de viata)
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• Primele manifestari clinice pot fi inselatoare– poliartrita febrila -simuland RAA-dar artritele
sunt moderate, persistente si fixe
– febra • izolata • asociata cu
– adenopatii– eruptii– splenomegalie
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FORMA CU DEBUT SISTEMICFORMA CU DEBUT SISTEMICTABLOU CLINICTABLOU CLINIC
• Febra – neregulata osciland intre 37-40o
• Eruptii cutanate
• Adenopatii
• Hepatosplenomegalie
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• Artrita adesea absenta in cursul primelor saptamani– bilateral si simetrica
– afecteaza mai frecvent pumnul, genunchii, gleznele
• Serozita– pericardita
– pleurezie
– serozita abdominala
• Miocardita rar• Intarzierea cresterii
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FORMA CU DEBUT SISTEMICFORMA CU DEBUT SISTEMICEXAMENE DE LABORATOREXAMENE DE LABORATOR
• Nu exista ex de laborator caracteristice• VSH crescut (50-100mm pt 1 h)• Proteina C reactiva – valori crescute• Hiperleucocitoza cu neutrofilie• Anemie normocroma normocitara• Trombocitoza• Serologia reumatoida e negativa
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FORMA CU DEBUT SISTEMICFORMA CU DEBUT SISTEMICEXAMEN RADIOLOGICEXAMEN RADIOLOGIC
• Tumefactia partilor moi, osteoporoza• Periostita • Imagini de distructie a cartilajului si a osului
subcondral
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EVOLUTIE, COMPLICATII, PROGNOSTICEVOLUTIE, COMPLICATII, PROGNOSTIC
• Evolutie
– in pusee inflamatorii febrile
– 25% evolueaza cu poliatrita severa
– puseu supra-acut cu miocardita, infectii – se poate pune in joc prognosticul vital
• Complicatii
– Sindromul de activare a macrofagelor (proliferare necontrolata a macrofagelor activate si eliberare de citokine proinflamatorii): anemie, leucopenie, trombocitopenie, tablou CID, cresterea transamin
– retard statural
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FORMA OLIGOARTICULARAFORMA OLIGOARTICULARA Sunt afectate cel mult 4 articulatii
• OligoartritaOligoartrita– persistenta – dupa primele 6 luni nu apar alte artritepersistenta – dupa primele 6 luni nu apar alte artrite
– extinsa – sunt afectate 5 sau mai multe art dupa extinsa – sunt afectate 5 sau mai multe art dupa primele 6 luni de boalaprimele 6 luni de boala
• Varsta de debut 1-6 ani - incidenta maxima la 2 ani• Debut
– manifestari articulare
– febra inconstanta si moderata
• Sunt afectate articulatii mari: genunchi, glezna, cot
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• Artritele sunt – asimetrice– evolueaza mai multe luni cu tumefactie artic.– rar distructive– pot apare tulburari de crestere
• Iridociclita cronica - cu semne si simptome discrete riscul cecitatii datorita dg tardiv
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• Ex laborator– hemograma si VSH – valori normale sau usor
modificate– FR neg– AAN poz– HLA DR-5, DR-8
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• lichidul articular – bogat in albumina– PMN– aseptic
• Ex histologic – sinovita nespecififica: – hipertrofia vilozitatilor– Hipervascularizatie– infiltrate celulare– depozite de fibrina
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• Complicatii– iridociclita se poate complica cu : glaucom,
sinechii, cataracta
• Evolutie – in pusee, dar prognosticul functional la distanta e relativ favorabil
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FORMA POLIARTICULARAFORMA POLIARTICULARA
• Sunt afectate mai mult de 4 articulatii in primele 6 luni de boala.
• E mai frecv la fete
• Debutul – febra moderata si afectare poliarticulara– monartrita prelungita
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• Artrita– simetrica– afecteaza
• art mici ale miinii
• art mari (genunchi, cot)
• art col cervicale
• art temporomandibulara
– redoare matinala
• Uneori manifestari sistemice: – febra moderata– anemie– leucocitoza
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FORMA POLIARTICULARA
• F seropozitiva pt FR– la 9-16 ani– dupa 1 an de evolutie cei mai multi au artrita
distructiva– HLA DR4
• Forma seronegativa pt FR
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EXAMEN RADIOLOGICEXAMEN RADIOLOGIC
• Leziuni importante si evolutive– tumefactia partilor moi
– osteoporoza
– periostita
– pensarea spatiului articular si neregularitati ale conturului osos
• Sunt afectate anumite teritorii:– art carpului
– art coxofemurala
– art col cervicale`
– art temporomandibulara
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• ARTRITA PSORIAZICAARTRITA PSORIAZICA = – artrita – +psoriazis = placi eritematoase, proeminente,
bine delimitate, acoperite de scuame albe-sidefii, usor detasabile
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• Entezita = inflamatia insertiilor osoase ale tendoanelor si ligamentelor. )
• Apare deobicei dupa varsta de 8 ani, • la baieti, • poate evolua ca spondilartropatie
• Afecteaza asimetric articulatiile membrelor inferioare– art genunchi– art coxofemurala– art tibiotarsiana– art metatarsofalangiana a halucelui
Artrita asociata enteziteiArtrita asociata entezitei
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• Astenie, uneori scadere ponderala, febra, anorexie
• Iridociclita acuta – scaderea acuitatii vizuale, eritem
• Ex lab – – Normale– uneori VSH crescut – HLA B27– FR si AAN neg
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• AINS– Acidul acetilsalicilic – 50-80 mg/Kg/zi– ibuprofen – 20-30 mg/Kg/zi– diclofenac – 2-4 mg/Kg/zi– naproxen 10-15 mg/kg/zi– Celecoxib (Celebrex) –inhibitori selectiv - COX-2 - >2 years:
>10 kg - <25 kg: 50 mg x 2/zi po>25 kg: 100 mg x 2/zi po
TRATAMENT
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• Corticoterapia– este recomandata:
• in formele sistemice necontrolate prin AINS• in formele sistemice cu poliserozita• iridociclita
– Metilprednisolon 15-30 mg/kg/zi – 2-3 zile– prednison 2mg/Kg/zi
• Metotrexat – 7,5 -15 mg/m2/sapt– efectele favorabile dupa 6-12 saptamini
TRATAMENT
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TRATAMENT
• Sulfasalazina - 40-60 mg/kg/zi – trat de intretinere= 30 mg/kg/zi
• Saruri de aur – 0,5 mg/Kg /saptamina 1mg/Kg/sapt
• D-penicilamina– 5 mg/Kg/zi 1 luna apoi 10-15 mg/Kg/zi– utilizare limitata in prezent
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• Agenti anti TNF – INFLIXIMAB– ETANERCEPT – 0,4 mg/kg/sapt
• Agenti care blocheaza IL-1 – ANAKINRA
• Anticorpi monoclonali antireceptori IL-6– MRA (tocilizumab)
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• Terapia afectarii oculare – midritice – corticoizi
• Corticoterapia intraarticulara – triamcinolon hexacetonid
• Sinovectomia are indicatii limitate la copil
• Kinetoterapie
TRATAMENT