幼年特发性关节炎.ppt

幼年特发性关节炎,复旦大学风湿过敏免疫中心 复旦大学附属儿科医院肾脏风湿科 周利军 ,Introduction,周四下午，风湿专科 54524666转5084 肾脏风湿科病房 54524666转3011 ,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15X109 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 CLASS a. Persistent arthritis of at least six weeks duration in one or more joints b. 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: Polyarthritis Oligoarthritis,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 uveitis Rheumatoid factor (RF) positive Seronegative, B27 positive Not otherwise classified,ACR Clinical Classification Criteria for Juvenile Rheumatoid Arthritis,*Patients with systemic onset JRA are excluded from this onset subtype. Polyarticular subtypes: RF positivity Not 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 2004,5