中国医科大学附属一院风湿免疫科张 榕.pptVIP

系统性红斑狼疮(SLE) Systemic Lupus Erythematosus 青帝 /read/0/296/ SLE onset by sex and age Etiology genetic factors environmental factors Sex hormonal factors Pathogenesis Excessive, abnormal production of “self” antibodies and formation of IC. Autoantibodies against nuclear, cytoplasmic and membrane components of multiple cell types in multiple organs . Pathology 光镜:1.结缔组织的纤维蛋白样变性 2.基质黏液性水肿 3.坏死性血管炎 特征性:疣状心内膜炎 苏木紫小体 “洋葱皮样”病变 临床应用：皮肤狼疮带试验 肾活检 skin and mucosa skin and mucosa skin and mucosa skin and mucosa skin and mucosa skin and mucosa Muscle and Joint arthritis --Jaccoud’s arthropathy Muscle pain、myositis femoral head necrosis Muscle and Joint lupus nephritis Class I 正常 Class II 系膜增殖性 mesangial Class III 局灶增殖性 focal proliferative Class IV 弥漫增殖性 diffuse proliferative Class V 膜性 membranous Class VI 肾小球硬化性glomerulosclerosis Nervous system clinical manifestation: -headache、vomiting -psychogenia -epilepsy -convulsion 、 conscious disturbance -coma pathology-vasculitis cerebrospinal fluid-no special (intracranial pressure↑protein↑cell population↑ glucose↓) Identify with other central nervous system disease lung 抗磷脂抗体综合征(antiphospholipid antibody syndrome) clinical manifestation: -arterous and/or venous thrombosis -spontaneous abortion -thrombocytopenia laboratory examination: -positive anti-phospholipid antibody 干 燥 综 合 征 30%的SLE患者可有继发干燥综合征 患者有临床症状 唾液腺ECT可有改变 干燥抗体可为阴性     提示SLE活动性的指证 症状体征:乏力、体重下降 发热 新出现的皮肤黏膜改变 关节炎 浆膜炎 尿少、浮肿 头痛、癜痫 辅助检查：血细胞减少 蛋白尿、血尿、管型尿、非感染性白细胞尿 补体下降 DNA抗体滴度升高 SLE病情轻重的评估 1.轻型SLE：无系统受累 2.重型SLE：有系统受累 3.狼疮危象(lupus crisis)：危及生命的重型SLE，包括：急进性狼疮肾炎，严重的中枢神经系统损害，严重的溶血性贫血，血