小儿风湿疾病RheumatoidDiseaseinChildren.pptVIP

小儿风湿性疾病Rheumatoid Disease in Children 浙江大学医学院附属儿童医院 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 Definition IgA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 skin Joint pain Gastrointestinal symptoms Renal 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 include Medications such as certain antibiotics, salicylic acid and thiazide diuretics) Pathogens bacterium/virus/parasite, such as group A strep, varicella, parvovirus and hepatitis B An acute respiratory infection Common allergens include Foods (such as berries, shellfish, fish, nuts, eggs, milk, and others) Pollen Animal dander (scales of shed skin) Insect bites Certain 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 体征 Palpable purpura, 尤其是下肢及臀部 Edema of the hands, feet, scalp and ears Arthritis,多发生于膝、踝、腕、肘等关节 腹部触痛 胃肠道出血 急性阴囊水肿 实验室检查 诊断要点 Diagnosis is clinical and not based on laboratory evaluation 多有感染、食物、药物、花粉、虫咬、疫苗接种等病史 有典型特征性皮肤紫癜，结合关节、胃肠或肾脏症状及反复发作史 有肾损害时，可见血尿及蛋白尿 血沉增快，CRP可阳性，IgA可增高 鉴别诊断 特发性血小板减少性紫癜 类风湿性关节炎 肠套叠 阑尾炎 肠梗阻 Treatment 原则 设法去除致敏因素 单纯者可用复方芦丁、钙剂、维生素C，抗组胺药 关节炎或消化道出血可用皮质类固醇激素 常用药物 抗过敏药物：开瑞坦、扑尔敏、葡萄糖酸钙等 降低血管通透性药物：vitC等 肾上腺皮质激素：氢化可的松、强的松、甲基强的松龙等 对于肾型或激素治疗不佳者：可用硫唑嘌呤、环磷酰胺等免疫抑制剂 预后 Prognosis is best in the absence of renal disease and neurologic involvement More than 80% of patients have a single isolated episode lasting a few weeks Approximately 10-20% of patients have recurrences Fewer than 5% of patients develop chronic HSP Abdominal pain resolves spontaneously within 72 ho