小儿风湿性疾病Rheumatoid Disease in Children XXX 医院 Connective tissue disease( 包含有120 种与关节、骨骼、肌肉等相关的疾病)过敏性紫癜 (anaphylactoid purpura)类风湿性关节炎 (JRA)风湿热 (rheumatic fever)川崎病 (kawasaki disease)系统性红斑狼疮 (systemic lupus erythematosis)皮肌炎 (dermatomyositis)硬皮病 (scleroderma)过敏性紫癜 Anaphylactoid purpura Henoch-Schonlein purpura Henoch-Schonlein syndrome(亨-舒综合征)DefinitionIgA 免疫复合物介导的白细胞碎裂性系统性小血管炎,病变主要累及皮肤、胃肠、关节及肾脏等部位Purple spots on the skinJoint painGastrointestinal symptomsRenal involvement General conditions较常见的微血管变态反应性出血性疾病春季上呼吸道感染后多发,也与昆虫叮咬或某些药物有关多发于2~8 岁的儿童男性较女性多见 病理毛细血管炎为主,亦可波及小静脉和小动脉血管壁可见胶原纤维肿胀和坏死,中性粒细胞浸润在皮肤和肾脏荧光显微镜下可见IgA 为主的免疫复合物沉积Common allergens includeMedications such as certain antibiotics, salicylic acid and thiazide diuretics)Pathogens bacterium/virus/parasite, such as group A strep, varicella, parvovirus and hepatitis BAn acute respiratory infectionCommon allergens includeFoods (such as berries, shellfish, fish, nuts, eggs, milk, and others)PollenAnimal dander (scales of shed skin)Insect bitesCertain vaccinations, such as for measles 临床表现皮肤症状:皮肤瘀点,对称分布、分批出现、大小不等、颜色深浅不一,可融合成片,多分布于臀部及下肢 关节症状:多发生在膝、踝、肘、腕等关节,关节腔可有渗液,但不留后遗症消化道症状:腹痛、呕吐,黑便或血便肾脏症状:肉眼血尿或镜下血尿、蛋白尿25 例过敏性紫癜的临床表现Purpura100%Arthritis75%Abdorminal pain65%Gastrointestinal bleeding30% Occult bleeding20% Gross bleeding10%Nephritis40% Microscopic haematuria10% Gross haematuria10% Proteinuria25% Nephrotic syndrome5% End-stage renal disease1%Recurrence of symptoms30%体征 Palpable purpura, ...