《红斑狼疮》ppt课件.ppt

* * 血浆置换 中草药：雷公藤多甙 干细胞移植 免疫基因治疗 其他治疗 * * 治疗策略 仅有实验室异常---严密观察、暂不用药 免疫学异常及面部红斑---抗疟药、必要时加用激素 免疫学异常及周身症状---小~中等量激素、必要时免疫抑制剂 内脏受累、血管炎---大剂量激素加免疫抑制剂 脏器严重受累、重度活动---激素冲击、免疫抑制剂 病情控制后---逐渐减少药物剂量、药物种类 病情缓解期---长期药物维持 * * 死因： 近期：狼疮活动，尤其是肾功衰竭、脑病、肺泡出血等；感染 远期：动脉粥样硬化、药物不良反应 不定期随诊、不遵医嘱、不规范治疗也是复发加重的原因。 预后 病例 1 女性，24岁 间断低热伴多关节痛3月 查体：双颊部红斑 血常规：白细胞计数 2.9×109/L ↓ 病例 2 女性，30岁 分娩后3天，头痛2天，突发意识障碍1天 既往史：妊娠后期颜面、双下肢水肿 查体：BP 160/100mmHg 浅昏迷 尿常规：红细胞 20-30个/HP 蛋白 +++ 腰穿：脑脊液 压力 210 cmH2O ↑ 蛋白 0.68g/L ↑ * 思考题 SLE属于哪类疾病？ SLE的基本病理机制是什么？ 狼疮性肾炎的病理分型及相应的临床特点。 神经精神性狼疮可以有哪些临床表现、脑脊液检查有何特点？ SLE治疗的原则是什么 ？ * 参考书目 Kelley’ s textbook of rheumatology 下卷 第74-76章 《中华风湿病学》 《临床诊疗指南》风湿病分册第八章 * * * 1 * 遗传因素 性激素和下丘脑垂体肾上腺轴 免疫学异常：自身抗体，免疫反应异常， 细胞因子网络，免疫调节缺陷，补体…… 环境因素：紫外线，药物，感染，饮食，社会心理因素等 * 1 * Discoid lupus erythematosus: Ig deposition, skin (photomicrograph) The typical fluorescent band is seen at the dermoepidermal junction (arrows) in discoid lupus. The band consists of granular deposits, which may be more densely or sparsely packed than those in this example. Deposits of immunoglobulin and complement at the dermoepidermal junction of lesional skin are a common finding in both systemic and chronic discoid lupus erythematosus. Similar deposits are frequently demonstrable in nonlesional skin of patients with systemic lupus but only rarely in patients with chronic discoid lupus. Ig deposits may also be found in other diseases. The fluorescence seen in this slide on the epidermal surface and in scattered dermal collagen bundles is a nonspecific finding. (cryostat section of unfixed skin, stained with fluorescein-conjugated anti-Ig; very high power) #9408040 * 1 * Systemic lupus erythematosus: IgG deposition, glomerulus (photomicrograph) The basement membrane of the glomerulus is partly outlined by irregular lumpy and bumpy deposits of antigen-antibody complex. These fluoresce apple green because they bind fluorescein-conjugated rabbit anti-serum ag