幼年特發(fā)性關(guān)節(jié)炎課件

幼年特發(fā)性關(guān)節(jié)炎復(fù)旦大學(xué)風(fēng)濕過敏免疫中心復(fù)旦大學(xué)附屬兒科醫(yī)院腎臟風(fēng)濕科周利軍fenlin183@.cn

幼年特發(fā)性關(guān)節(jié)炎Introduction周四下午，風(fēng)濕?？?4524666轉(zhuǎn)5084腎臟風(fēng)濕科病房54524666轉(zhuǎn)3011fenlin183@.cn幼年特發(fā)性關(guān)節(jié)炎幼年特發(fā)性關(guān)節(jié)炎（JIA)JuvenileIdiopathicArthritisJuvenileRheumaticArthritisJuvenileChronicArthritis

2001,JIAhasbeenconfirmedbyILAR幼年特發(fā)性關(guān)節(jié)炎TypesofAutoimmuneDisease幼年特發(fā)性關(guān)節(jié)炎幼年特發(fā)性關(guān)節(jié)炎（JIA)16歲以下兒童長期發(fā)熱、皮疹淋巴結(jié)和肝脾腫大胸膜炎和心包炎反復(fù)發(fā)作可致關(guān)節(jié)畸形幼年特發(fā)性關(guān)節(jié)炎幼年特發(fā)性關(guān)節(jié)炎（JIA)3歲男孩，弛張熱，肝脾腫大少量心包積液、充血性隨熱出現(xiàn)皮疹疾?。?？？幼年特發(fā)性關(guān)節(jié)炎病因和發(fā)病機(jī)理感染遺傳1979StastyFinkHLA-DW7,DW8寒冷\潮濕疲勞\營養(yǎng)不良幼年特發(fā)性關(guān)節(jié)炎病因和發(fā)病機(jī)理幼年特發(fā)性關(guān)節(jié)炎病理變化關(guān)節(jié)病變

皮下結(jié)節(jié)

眼部病變

其他幼年特發(fā)性關(guān)節(jié)炎免疫異常和損傷依據(jù)血IgGIgAIgM不同程度升高部分患兒補(bǔ)體升高可出現(xiàn)血和關(guān)節(jié)液RF陽性血和關(guān)節(jié)液TNF

水平增高遲發(fā)超敏反應(yīng)降低幼年特發(fā)性關(guān)節(jié)炎自身抗體的臨床價值在典型風(fēng)濕病—

肯定診斷

（A-dsDNA,ACL)在不典型風(fēng)濕病—

提示診斷如多關(guān)節(jié)炎(CCP抗體陽性)評估預(yù)后及指導(dǎo)用藥抗體譜廣+高滴度病情重用藥規(guī)范幼年特發(fā)性關(guān)節(jié)炎自身抗體的臨床價值幼年特發(fā)性關(guān)節(jié)炎新近JIA相關(guān)抗體抗CCP抗體BiP抗體AFA抗GPI抗體抗CB10抗體幼年特發(fā)性關(guān)節(jié)炎臨床表現(xiàn)Manifestation全身型多關(guān)節(jié),RF+多關(guān)節(jié),RF-少關(guān)節(jié),持續(xù)或擴(kuò)展

銀屑病型

附著點(diǎn)關(guān)節(jié)炎型其他幼年特發(fā)性關(guān)節(jié)炎全身型Systemiconset急性發(fā)病多見于2~4歲幼兒，JIA1/5~1/4反復(fù)弛張熱隨熱出現(xiàn)的一過性紅色斑疹胸膜炎、心包炎淋巴結(jié)及肝脾腫大白細(xì)胞計數(shù)>15X109貧血和血小板升高發(fā)熱先于關(guān)節(jié)癥狀幼年特發(fā)性關(guān)節(jié)炎多關(guān)節(jié)型受累關(guān)節(jié)>5個RF陰性和陽性亞型關(guān)節(jié)梭形腫脹關(guān)節(jié)積液、晨僵RF及ANA陽性關(guān)節(jié)病變嚴(yán)重幼年特發(fā)性關(guān)節(jié)炎少關(guān)節(jié)型是較多見的JIA受累關(guān)節(jié)<4個主要累及大關(guān)節(jié)RF陰性可發(fā)生虹膜睫狀體炎RF陽性可轉(zhuǎn)化為強(qiáng)直性脊柱炎,HLA-B27陽性幼年特發(fā)性關(guān)節(jié)炎免疫檢測血IgGIgMIgA增高血ANARF可異常

外周血CD4/CD8

血HLA-B27（排除診斷強(qiáng)直性脊柱炎，并詢問一級親屬家族史）幼年特發(fā)性關(guān)節(jié)炎實(shí)驗(yàn)室檢查血液檢查(類白血病，貧血，血小板明顯升膏）免疫檢測（series,CCP)關(guān)節(jié)腔積液檢查（關(guān)節(jié)穿刺）骨髓細(xì)胞學(xué)檢查(一般,危重)X線檢查(SHARPEVALUATION)MRI(TYPICAL)關(guān)節(jié)鏡檢查(骨中心）幼年特發(fā)性關(guān)節(jié)炎關(guān)節(jié)鏡檢查幼年特發(fā)性關(guān)節(jié)炎關(guān)節(jié)鏡檢查幼年特發(fā)性關(guān)節(jié)炎關(guān)節(jié)鏡檢查幼年特發(fā)性關(guān)節(jié)炎影象評價：病情評估

sharp評分幼年特發(fā)性關(guān)節(jié)炎血液檢查白細(xì)胞升高血小板升高貧血血沉加快CRP升高粘蛋白升高幼年特發(fā)性關(guān)節(jié)炎診斷（ACR)<16ysArthritis關(guān)節(jié)炎6monssubtypeanalysisAccordingtonewsubtypes(JIA)應(yīng)做鑒別診斷幼年特發(fā)性關(guān)節(jié)炎

ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritis

GENERALCLASS

a.Persistentarthritisofatleastsixweeksdurationinoneormorejoints

b.Exclusionofothercausesofarthritis(seelistofexclusions+)onsetsubtypes-determinedbymanifestationsduringthefirstsixmonthsofdiseasealthoughmanifestationsmorecloselyresemblinganothersubtypemayappearlaterSystemiconsetJRA*

subtypes:

Polyarthritis

Oligoarthritis幼年特發(fā)性關(guān)節(jié)炎ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritis*TypicalfeverandrashwillbeconsideredprobablesystemiconsetJRAifnotassociatedwitharthritis.Beforeadefinitediagnosiscanbemade,arthritis,asdefinedmustbepresent.Pauciarticular*

subtypes:

Antinuclearantibody(ANA)positive-chronicuveitis

Rheumatoidfactor(RF)positive

Seronegative,B27positive

Nototherwiseclassified幼年特發(fā)性關(guān)節(jié)炎ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritis*PatientswithsystemiconsetJRAareexcludedfromthisonsetsubtype.Polyarticular

subtypes:

RFpositivity

Nototherwiseclassified*PatientswithsystemicJRAonsetareexcludedfromthissubtype.幼年特發(fā)性關(guān)節(jié)炎ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritis

OtherrheumaticdiseasesRheumaticfeverSystemiclupuserythematosusAnkylosingspondylitisPolymyositisordermatomyositisVasculiticsyndromesSclerodermaPsoriaticarthritisReiter'ssyndromeSjogren'ssyndromeMixedconnectivetissuediseaseBehcet'ssyndrome幼年特發(fā)性關(guān)節(jié)炎ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritisInfectiousarthritisInflammatoryboweldiseaseNeoplasticdiseasesincludingleukemiaNonrheumaticconditionsofbonesandjointsHematologicdiseasesPsychogenicarthralgiaMiscellaneousSarcoidosisHypertrophicosteoarthropathyVillonodularsynovitisChronicactivehepatitisFamilialMediterraneanfever幼年特發(fā)性關(guān)節(jié)炎ACRClinicalClassificationCriteriaforJuvenileRheumatoidArthritisReference:JRACriteriaSubcommitteeoftheDiagnosticandTherapeuticCriteriaCommitteeoftheAmericanRheumatismAssociationArthritisRheum20(Suppl)195,1977幼年特發(fā)性關(guān)節(jié)炎ACRGuidelinesforMedicalManagementofRheumatoidArthritis(updatedApril,2002)

幼年特發(fā)性關(guān)節(jié)炎鑒別診斷

感染性關(guān)節(jié)炎風(fēng)濕性關(guān)節(jié)炎系統(tǒng)性紅斑狼瘡過敏性紫癜絨毛膜色素沉著性關(guān)節(jié)炎急性白血病其他血管炎綜合征幼年特發(fā)性關(guān)節(jié)炎ComplicationGrowthfailureMASServerinfectionTBinfectionSideeffectsbysteriodandimmuneinhibitorandbioagents幼年特發(fā)性關(guān)節(jié)炎JIA治療目標(biāo)幼年特發(fā)性關(guān)節(jié)炎治療

傳統(tǒng)抗炎藥物治療病程緩解藥免疫抑制劑新近治療生物制劑造血干細(xì)胞移植血液凈化展望治療異基因移植治療幼年特發(fā)性關(guān)節(jié)炎幼年特發(fā)性關(guān)節(jié)炎抗炎藥物治療非甾體類藥物(NASID)布洛芬扶他林尼美舒利萘普生阿司匹林以上藥物FDA通過可在兒童應(yīng)用

幼年特發(fā)性關(guān)節(jié)炎布洛芬1964年由英國Boots分司開發(fā)成功1995年美國FDA批準(zhǔn)布洛芬混懸液為OTC1989年布洛芬緩釋膠囊進(jìn)入中國幼年特發(fā)性關(guān)節(jié)炎病情緩解藥(DMARD)甲氨蝶呤來氟米特金制劑青霉胺羥氯喹柳氮磺胺吡啶幼年特發(fā)性關(guān)節(jié)炎羥氯喹作用于抗原呈遞環(huán)節(jié)幼年特發(fā)性關(guān)節(jié)炎羥氯喹作用羥基氯喹對早期類風(fēng)濕關(guān)節(jié)炎患者癥狀控制有效，（E.L.Matteson,Rheumatology2004;43:619-625）對影像學(xué)改變的影響與其他慢作用藥相似（JorgJ.Goronzy,Arthr﹠Rheum2004,50(1);43-54）幼年特發(fā)性關(guān)節(jié)炎羥氯喹作用幼年特發(fā)性關(guān)節(jié)炎羥氯喹作用羥氯喹不減少幼年特發(fā)性關(guān)節(jié)炎患兒的MTX血藥濃度羥氯喹與MTX