幼特发性关节炎.ppt

,单击此处编辑母版标题样式,*,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,Introduction,周四下午，风湿专科,54524666,转,5084,肾脏风湿科病房,54524666,转,3011,fenlin183,幼年特发性关节炎（,JIA),Juvenile Idiopathic Arthritis,Juvenile Rheumatic Arthritis,Juvenile Chronic Arthritis,2001,JIA has been confirmed by ILAR,Types of Autoimmune Disease,幼年特发性关节炎（,JIA),16,岁以下儿童,长期发热、皮疹,淋巴结和肝脾肿大,胸膜炎和心包炎,反复发作可致关节畸形,幼年特发性关节炎（,JIA),3,岁男孩，弛张热，肝脾肿大,少量心包积液、充血性随热出现,皮疹,疾病？,病因和发病机理,感染,遗传,1979 Stasty Fink,HLA-DW7,DW8,寒冷,潮湿,疲劳,营养不良,病因和发病机理,病理变化,关节病变,皮下结节,眼部病变,其他,免疫异常和损伤依据,血,IgG IgA IgM,不同程度升高,部分患儿补体升高,可出现血和关节液,RF,阳性,血和关节液,TNF,水平增高,迟发超敏反应降低,自身抗体的临床价值,在典型风湿病,肯定诊断（,A-dsDNA,ACL),在不典型风湿病,提示诊断,如多关节炎,(CCP,抗体阳性,),评估预后及指导用药,抗体谱广,+,高滴度 病情重,用药规范,自身抗体的临床价值,新近,JIA,相关抗体,抗,CCP,抗体,BiP,抗体,AFA,抗,GPI,抗体,抗,CB10,抗体,临床表现,Manifestation,全身型,多关节,RF+,多关节,RF-,少关节,持续或扩展,银屑病型,附着点关节炎型,其他,全身型,Systemic onset,急性发病,多见于,24,岁幼儿，,JIA 1/51/4,反复,弛张热,随热出现的,一过性红色斑疹,胸膜炎、,心包炎,淋巴结及肝脾肿大,白细胞计数,15X10,9,贫血和血小板升高,发热先于关节症状,多关节型,受累关节,5,个,RF,阴性和阳性亚型,关节梭形肿胀,关节积液、晨僵,RF,及,ANA,阳性关节病变严重,少关节型,是较多见的,JIA,受累关节,4,个,主要累及大关节,RF,阴性可发生,虹膜睫状体炎,RF,阳性可转化为,强直性脊柱炎,HLA-B27,阳性,免疫检测,血,IgG IgM IgA,增高,血,ANA RF,可异常,外周血,CD4/CD8,血,HLA-B27,（排除诊断强直性脊柱炎，并询问一级亲属家族史,）,实验室检查,血液检查,(,类白血病，贫血，血小板明显升膏）,免疫检测（,series,CCP),关节腔积液检查（关节穿刺）,骨髓细胞学检查,(,一般,危重,),X,线检查,(SHARP EVALUATION),MRI(TYPICAL),关节镜检查,(,骨中心）,关节镜检查,关节镜检查,关节镜检查,影象评价：病情评估,sharp,评分,血液检查,白细胞升高,血小板升高,贫血,血沉加快,CRP,升高,粘蛋白升高,诊断（,ACR),16ys,Arthritis,关节炎,6mons subtype analysis,According to new subtypes(JIA),应做鉴别诊断,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,GENERAL CLASSa.Persistent arthritis of at least six weeks duration in one or more jointsb.Exclusion of other causes of arthritis(see list of exclusions+),onset subtypes-determined by manifestations during the first six months of disease although manifestations more closely resembling another subtype may appear later,Systemic onset JRA*subtypes:PolyarthritisOligoarthritis,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,*Typical fever and rash will be considered probable systemic onset JRA if not associated with arthritis.Before a definite diagnosis can be made,arthritis,as defined must be present.,Pauciarticular*subtypes:Antinuclear antibody(ANA)positive-chronic uveitisRheumatoid factor(RF)positiveSeronegative,B27 positiveNot otherwise classified,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,*Patients with systemic onset JRA are excluded from this onset subtype.,Polyarticularsubtypes:RF positivityNot otherwise classified,*Patients with systemic JRA onset are excluded from this subtype.,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,Other rheumatic diseases,Rheumatic fever,Systemic lupus erythematosus,Ankylosing spondylitis,Polymyositis or dermatomyositis,Vasculitic syndromes,Scleroderma,Psoriatic arthritis,Reiters syndrome,Sjogrens syndrome,Mixed connective tissue disease,Behcets syndrome,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,Infectious arthritis,Inflammatory bowel disease,Neoplastic diseases including leukemia,Nonrheumatic conditions of bones and joints,Hematologic diseases,Psychogenic arthralgia,Miscellaneous,Sarcoidosis,Hypertrophic osteoarthropathy,Villonodular synovitis,Chronic active hepatitis,Familial Mediterranean fever,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,Reference:JRA Criteria Subcommittee of the Diagnostic and Therapeutic Criteria Committee of the American Rheumatism Association Arthritis Rheum 20(Suppl)195,1977,ACR Guidelines for Medical Management of Rheumatoid Arthritis(updated April,2002),鉴别诊断,感染性关节炎,风湿性关节炎,系统性红斑狼疮,过敏性紫癜,绒毛膜色素沉着性关节炎,急性白血病,其他血管炎综合征,Complication,Growth failure,MAS,Server infection,TB infection,Side effects by steriod and immune inhibitor and bioagents,JIA,治疗目标,治疗,传统,抗炎药物治疗,病程缓解药,免疫抑制剂,新近治疗,生物制剂,造血干细胞移植,血液净化,展望治疗,异基因移植治疗,抗炎药物治疗,非甾体类药物,(NASID),布洛芬,扶他林,尼美舒利,萘普生,阿司匹林,以上药物,FDA,通过可在儿童应用,布洛芬,1964,年由英国,Boots,分司开发成功,1995,年美国,FDA,批准布洛芬混悬液为,OTC,1989,年布洛芬缓释胶囊进入中国,病情缓解药,(DMARD),甲氨蝶呤,来氟米特,金制剂,青霉胺,羟氯喹,柳氮磺胺吡啶,羟氯喹作用于抗原呈递环节,羟氯喹作用,羟基氯喹对早期类风湿关节炎患者症状控制有效，（,E.L.Matteson,Rheumatology 2004;43:619-625,）,对影像学改变的影响与其他慢作用药相似（,Jorg J.Goronzy,Arthr Rheum 2004,50(1);43-54,）,羟氯喹作用,羟氯喹作用,羟氯喹不减少幼年特发性关节炎患儿的,MTX,血药浓度,羟氯喹与,MTX,联用是有效、安全的,JRA,治疗方案,R 25(5):1621-2,激素,全身治疗,眼科治疗,关节腔治疗,其他免疫抑制剂,AZA,CTX,CSA,MMF,其他新型免疫抑制剂,酶酚酸酯,MMF,MPA,esterases,总结,长期随访，规则治疗,循证原则,选择安全有效的药物治疗,进行统一的评价体系分析,个体化治疗,合理诱导、巩固和维持治疗,新医院,展望未来,资料可以编辑修改使用,学习愉快！,课件仅供参考哦，,实际情况要实际分析哈！,The user can demonstrate on a projector or computer,or print the presentation and make it into a film to be used in a wider field,感谢您的观看,