幼年性类风湿性关节炎(JRA Juvenile rheumatoid arthritis 儿科医院邬惊雷
幼年性类风湿性关节炎 (JRA) Juvenile Rheumatoid Arthritis 儿科医院 邬惊雷
是一种少年儿童常见的结缔组织病 One of the most common connective tissue diseases of children 以慢性关节炎(滑膜炎)为主要特点 Characterized by chronic arthritis(synovitis of the joints) 多脏器受累,如肝脾肿大、淋巴结肿大、浆膜 炎(心包积液)等 Visceral involvement such as hepatosplenomegaly, lym phadenopathy and serositis(pericardial effusion)
是一种少年儿童常见的结缔组织病 One of the most common connective tissue diseases of children 以慢性关节炎(滑膜炎)为主要特点 Characterized by chronic arthritis (synovitis of the joints) 多脏器受累，如肝脾肿大、淋巴结肿大、浆膜 炎(心包积液)等 Visceral involvement ,such as hepatosplenomegaly, lymphadenopathy and serositis (pericardial effusion)
分类 Classification 全身型 systemic-onset disease 多关节型:5个或以上关节受累 Polyarthritis: 5 or more inflamed joints 少关节型:5个以下关节受累 Oligoarthritis: <5 inflamed joints
分类 Classification 全身型 systemic-onset disease 多关节型:：5个或以上关节受累 Polyarthritis:5 or more inflamed joints 少关节型：5个以下关节受累 Oligoarthritis: < 5 inflamed joints
流行病学 Epidemiology 发病率约113/10万 Overall prevalence approximately 113/100,000 约占住院0.1%-0.15% Accounts for about 0. 1%0-0.15% hospitalizations 女性发病较多 Females affected more frequently than males
流行病学Epidemiology 发病率约113/10万 Overall prevalence approximately 113/100,000 约占住院0.1%-0.15% Accounts for about 0.1%-0.15% hospitalizations 女性发病较多 Females affected more frequently than males
病因 Etiology 病因尚不清楚,可能与下列因素有关 Etiology is unknown, maybe related to the follows 免疫遗传易感性 Immunogenetic suseptibility 外源性触发因素(病毒感染) External triggers (virus infection)
病因 Etiology 病因尚不清楚,可能与下列因素有关: Etiology is unknown，maybe related to the follows: 免疫遗传易感性 Immunogenetic suseptibility 外源性触发因素 (病毒感染） External triggers （virus infection)
易感儿童在感染(环境因素)作用下出现免疫 异常的结果 Exaggerated immune reactivity in predisposed children in response to exposure to certain infections(environmental factors)
易感儿童在感染（环境因素）作用下出现免疫 异常的结果 Exaggerated immune reactivity in predisposed children in response to exposure to certain infections (environmental factors)
病理变化 Pathologic changes 关节:绒毛状增厚为特征的滑膜炎 Synovitis characterized by villous hypertrophy 滑膜下组织充血、水肿 Hyperemia and edema of the subsynovial tissues 血管翳形成 Pannus formation 关节软骨和邻近骨组织的破坏 Erosion of articular cartilage and contiguous bone
病理变化Pathologic changes 关节：绒毛状增厚为特征的滑膜炎 Synovitis characterized by villous hypertrophy 滑膜下组织充血、水肿 Hyperemia and edema of the subsynovial tissues 血管翳形成 Pannus formation 关节软骨和邻近骨组织的破坏 Erosion of articular cartilage and contiguous bone
皮下结节: Subcutaneous( Rheumatoid) nodules 见于肘关节伸侧面 Found on extensor surfaces of elbows 眼部病变 虹膜睫状体炎,尤见于少关节型病例 iridocyclitis mainly in the cases of oligoarthritis
皮下结节：Subcutaneous (Rheumatoid) nodules 见于肘关节伸侧面 Found on extensor surfaces of elbows 眼部病变 虹膜睫状体炎，尤见于少关节型病例 Iridocyclitis ,mainly in the cases of oligoarthritis
临床表现 Clinical manifestations 1全身型:占10%-20%JRA病例 Systemic-onset disease, 10%-20%cases 发热:呈弛张热型是此型特点,体温高峰达40 热退时一般情况好 Fever: Characterized by a remmitent fever with temperature spikes to 40C. Present well when fever going down
临床表现Clinical manifestations 1.全身型 ：占10％-20％JRA病例 Systemic-onset disease，10％-20％ cases 发热：呈弛张热型是此型特点，体温高峰达40oC, 热退时一般情况好 Fever: Characterized by a remmitent fever with temperature spikes to 40oC .Present well when fever going down
41 40 肛门体温C 39 38 37 36 Ta 3岁JRA女孩 发烧多天 经反复应用退烧药无效
肛 门 体 温 oC 3 岁JRA女孩： －发烧多天 －经反复应用退烧药无效