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天疱疮研究的某些新进展精品课件_1

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天疱疮研究的某些新进展
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Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮研究的某些新进展 西安交通大学第二医院 冯捷 徐汉卿 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮定义 天疱疮是一类以角朊细胞粘着性丧 失,棘层松懈,皮肤粘膜出现松驰大疱 为特征的自身免疫性疾病,所有天疱疮 都与组织内或循环中出现的抗表皮细胞 间抗原的自身抗体有关。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 1、 天疱疮抗原 I (1)天疱疮有关的细胞间粘 连分子——跨膜桥粒蛋白, 又称桥粒核心蛋白,具有调 节桥粒内跨膜粘连分子的功 能。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮抗原 II (2)寻常型天疱疮抗原 免疫沉淀反应测出所有寻常 型天疱疮抗原血清,均能与表 皮内210-130-85KD的抗原复 合物,测出寻常型天疱疮抗原 。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 2、天疱疮的免疫活性细胞 活性T淋巴细胞,单核细胞、 巨噬细胞也参与天疱疮病变形 成。天疱疮疱液中,外周血中 IL-2R的水平升高;在接受治疗 时随着IL-2R水平下降和临床症 状好转相关。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 3、天疱疮抗体(抗棘细胞抗体 ) (1) 天疱疮患者血清中存在 天疱疮抗体。 (2) 该抗体与病情的严重程 度相平行,高于1:40。 (3) 该抗体是病原。 (4) 反映临床症状的改变。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮病因与诱因: (1)自身免疫性疾病 所有活动的天疱疮患者血清中有 天疱疮抗体——IgG、IgM、IgA 滴度与病情呈正相关。 (2)诱因:发烧、感染、药物( 长效青霉素胺,保泰松,利福平 )。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮临床分型 (1)寻常型天疱疮及其变型增殖 型天疱疮(2)落叶型天疱疮及其变 型红斑型天疱疮(3)药物诱导的天 疱疮(4)IgA天疱疮(5)副肿瘤性天 疱疮(6)疱疹样天疱疮。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮组织学特征及免疫学检查 (1) 皮内水疱及嗜酸性,嗜中性混 合炎症细胞浸润。 (2) 由于组织学特征与其它一些疾 病相类似,因此需要直接(DIF 免疫 )荧光和间接免疫荧光(IIF)检查。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 直接免疫荧光(DIF) 检查阳性呈现:IgG和或C3沿角朊细胞 膜呈波纹状沉积。由于在其它一些自身 免疫性大疱性疾病中也可有类似表现。 因此,还必须进行表皮提取物的蛋白免 疫沉淀和免疫印迹,可明确靶抗原的分 子量。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮进一步检查确诊: (1) 组织学鉴定。 (2) 皮肤组织进行盐裂DIF检测 ,或通过免疫电镜——明确反应 物的定位。 (3) 每一表皮内自身免疫性大疱 性皮肤病均可通过自身免疫的靶 抗原加以定义——IIF间接免疫荧 光。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮诊断标准: (1) 临床皮肤和粘膜的疱性糜烂 。 (2) 组织学:基层上方棘层松懈 性水疱。 (3) DIF:受累处表皮细胞表面 IgG、C3、IgA沉积。 (4) IIF:循环抗体,滴度1:40 以上。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development.天疱疮治疗的目的 (1) 减少自身抗体的合成是治疗 的目的。 (2) 抗体合成多少的表现——抗 表面抗体滴度。 (3) 滴度与病情严重程度呈高度 正相关。活动性病变时——高滴度 抗体;病情缓解时较低或检测阴性 。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮治疗: (1)皮质类固醇(CS) (2)免疫抑制剂(CS1) (3)免疫球蛋白(IVIg) (4)血浆置换(PMR) (5)地塞米松——CTX冲击疗法 (6)体外光化学疗法(ECP) (7) 其它疗法 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 皮质类固醇与免疫抑制剂并用 : (1) CS1可使CS迅速减量; 避免长期大量使用CS的副作用 ,改善预后。 (2) 常用免疫抑制剂:环磷 酰胺(CTX),环孢素A,硫 唑嘌呤。 *但甲氨蝶呤(MTX)不宜使用 。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 环磷酰胺(CTX) 作用:对增殖的浆细胞有优先 毒性作用,可有效的减少自身 抗体合成。 付作用:白细胞减少,不育, 出血性膀胱炎和恶性肿瘤的终 生危险性增加。 方法:激素+CTX:1~2mg/kg/d Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 环孢素A与硫唑嘌呤 环孢素 A与激素合并应用可取得较 好的效果,潜在的肾毒性妨碍了其应 用5~8mg/kg/d,分2次口服,病情控 制后减为2~3mg/kg/d,可连续服1~ 2年。 硫唑嘌呤:其毒性低于CTX,但其 疗效亦差,2~3mg/kg/d分次口服, 联用小量强的松。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. PMR与CS与CS1同时并用优点: (1) PMR能迅速清除天疱疮抗 体, 但由于机体免疫网络中的反 馈机制将天疱疮抗体于PMR之后 迅速回升,甚至超过治疗前水平 。因此,PMR必须配合强有力的 免疫抑制剂,抑制新抗体的产生 。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development.PMR与CS与CSi并用 (2) 适应症:常规CS1无效的重症病例 。 (3) 副作用:虽PMR疗效显著,但可以 导致患者电解质紊乱,肺水肿,出血,休 克,甚至死亡。 (4) 方法:PMR+CTX500mg/d×3天, 三天后,CTX采用常规剂量,CS迅速减量 ,10个月后停全部用药,完全缓解。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 免疫球蛋白静脉注射+CS与CS1同时并用 I IvIg治疗天疱疮的机理: (1) IVIg中的抗独特型抗体具有特异的抗 原结合能力,有效中和致病抗体。 (2) 网状内皮系统清除自身抗体的过程加 快。 (3) 与特异性B细胞受体结合,受体功能 下调,抗体合成减少。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. IVIg+CS 与CSI同时并用 II 适应症:用CSI常规治疗难以 控制的严重的天疱疮病例。 方法:IVIg 400mg/kg/d ×5天 CS 2mg/kg/d 硫唑嘌呤 100mg--150mg/d Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法三大优点Ⅰ (1) 迅速控制病情,经济,方便 ,不需住院,每月一次在门诊接受 DCP,2~3小时即可离开。 (2) 安全可靠,治疗过程中无严 重副作用。 (3) 可使天疱疮获得持久性缓解 。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) II 适应症:用常规CSI不能控制 病情的天疱疮。 疗效:随着治疗时间的延长, 天疱疮抗体滴度逐渐下降乃至 消失,CS副作用很轻,或缺如 ,长期缓解。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) III 实施方案:第一步 (1) 每月一次DCP,直至皮损完 全消失。 (2) 间歇期 CTX 50mg/d (3) 如在接收 DCP前已用CS, 则DCP后逐渐减量至停药 仅用CTX 500mg/d Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) Ⅳ 实施方案:第二步 (1) 皮损完全消失后再给DCP 至少6次。 (2) 间歇期仍给予CTX 50mg/d (即半年时间) Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) Ⅴ 实施方案:第三步 (1) 如果患处于缓解期,则停 用DCP。 (2) 仅口服CTX 50mg/d,持续 一年。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) Ⅵ 实施方案:第四步 (1) 患者处于缓解期停用CTX。 (2) 随访、观察。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. CS冲击疗法(DCP) Ⅶ 传统冲击疗法: 甲泼尼龙冲击疗法1250mg/d ,连用3~5天。可产生严重的 副作用, 甚至可致死,目前已 不采用。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 新的CS击疗法(DCP) Ⅷ 地塞米松冲击疗法 地塞米松100mg/d×3天 同时CTX 500mg/d静点, 连续3日,每月一次。间歇期 CTX 500mg/d。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮其它疗法 Ⅰ (1) 光化学疗法(ECP)+CSI ECP:口服补骨脂素0.6mg/kg, 2小时后, 取富含白细胞的血液接受 长波紫外线照射,然后回输体内。每 月2次。 (2) 前腺素E:0.5mg Bid。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮其它疗法 Ⅱ (3) 肝素:肝素能抑制T、B淋 巴细胞的玫瑰花环形成,减轻抗 体靶细胞的毒性,并抑制T-B淋巴 细胞之间的协作。 肝素单独使用有效,而与CS联 合应用疗效较好。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮的其它疗法 Ⅲ (4) 雷公藤多甙(40~60mg/d )与强的松联用。 (5) 氨苯砜(DDS) 50~100mg/d,与强的松联用。 (6) 磺胺吡碇(2~3g/d)与强 的松联用。 (7) 左旋咪唑 0.1~0.2/d,与强 的松联用。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮的预后 Ⅰ 无口服糖皮质激素制剂的20世纪50 年代,本病的死亡率分别为2年50% , 5 年100%。死亡的主要原因是因 为衰弱、电解质及蛋白质丧失、摄食 减少和感染、脓毒血症。目前的死亡 率仅为5%,免疫抑制剂治疗的并发症 ,几乎是唯一的死亡原因。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development. 天疱疮的其它疗法 IV 金制剂----硫代苹果酸全钠(gold sodium thiomalate)每周一次, 肌 肉注射,首次10mg,第二次数25mg ,自第三周起每周50mg。 直至病情 稳定后改为2~4周一次,肌肉注射 50mg(300mg起效) 付作用:过敏。 Through this publicity campaign, further improve the sense of business integrity of tourism enterprises to help tourism consumers and operators to safeguard the legitimate rights and interests, to ensure that our City's tourism sustainable, healthy and coordinated Development.天疱疮的预后 II 综述天疱疮10年来治疗研究进展,皮质 类固醇与免疫制剂同时应用,极大地改善 了天疱疮的预后,在此基础上配合血浆置 换疗法,改良的冲击疗法,及大剂量免疫 球蛋白静脉注射,使难治性天疱疮得到有 效的控制甚至于长期缓解。随着传统药物 (疗法)的最佳组合及新药物,新疗法的 不断问世,治愈天疱疮的希望将成为现实 。 单击此处编辑母版标题样式 单击此处编辑母版副标题样式 *36 rXiOaF1w$nTeK6B)sYjPbG2x%oUgL7C-tZlQcH3y&qVhM8D+u#mRdI4z*rWiN9E0w!nSeJ5B(sXjOaG1x$oTfL6C)tYkPbH2y%pUgM7D-uZlRcI3z&qWhN8E+v!mSdJ4A(rXiO9F0w$nTeK5B)sYjPaG2x%oUfL7C-tZkQcH3y&pVgM8D+u#lRdI4z*qWiN9E0v!nSeJ5A(sXjOaF1x$oTfK6B)tYkPbG2y%pUgL7D-uZlQcI3z&qVhN8E+v#mRdJ4A*rWiO9F0w!nTeK5B(sYjPaG1x%oUfL6C-tZkQbH2y&pVgM7D+u#lRcI4z*qWhN9E0v!mSeJ5A(rXjOaF1w$nTfK6B)sYkPbG2x%pUgL7C-uZlQcH3z&qVhM8D+v#mRdI4A*rWiN9F0w!nSeK5B(sXjPaG1x$oUfL6C)tYkQbH2y%pVgM7D-u#lRcI3z*qWhN8E0v!mSdJ4A(rXiO9F1w$nTeK6B)sYjPbG2x%oUgL7C- tZlQcH3y&pVhM8D+u#mRdI4z*rWiN9E0w!nSeJ5B(sXjOaF1x$oTfK6C)tYkPbH2y%pUgM7D-uZlRcI3z&qWhN8E+v#mSdJ4A*rXiO9F0w$nTeK5B)sYjPaG2x%oUfL7C-tZkQbH3y&pVgM8D+u#lRdI4z*qWiN9E0v!nSeJ5A(rXjOaF1w$oTfK6B)tYkPbG2y%pUgL7D-uZlQcI3z&qVhM8E+v#mRdJ4A*rWiO9F0w!nTeK5B(sYjPaG1x$oUfL6C)tZkQbH2y&pVgM7D+u#lRcI4z*qWhN9E0v!mSdJ5A(rXiOaF1w$nTfK6B)sYkPbG2x%pUgL7C-uZlQcH3y&qVhM8D+v#mRdI4A*rWiN9F0w!nSeK5B(sXjOaG1x$oTfL6C)tYkQbH2y%pVgM7D-u#lRcI3z*qWhN8E+v!mSdJ4A(rXiO9F1w$nTeK6B)sYjPbG2x%oUfL7C- tZkQcH3y&pVhM8D+u#mRdI4z*rWiN9E0w!nSeJ5A(sXjOaF1x$oTfK6C)tYkTeK5B(sYjPaG1x%oUfL6C-tZkQbH3y&pVgM8D+u#lRcI4z*qWhN9E0v!mSeJ5A(rXjOaF1w$oTfK6B)sYkPbG2x%pUgL7C-uZlQcH3z&qVhM8E+v#mRdJ4A*rWiN9F0w!nSeK5B(sXjPaG1x$oUfL6C)tZkQbH2y&pVgM7D-u#lRcI3z*qWhN8E0v!mSdJ5A(rXiOaF1w$nTeK6B)sYjPbG2x%oUgL7C-tZlQcH3y&qVhM8D+v#mRdI4z*rWiN9E0w!nSeJ5B(sXjOaG1x$oTfL6C)tYkPbH2y%pUgM7D-uZlRcI3z&qWhN8E+v!mSdJ4A(rXiO9F0w$nTeK5B)sYjPaG2x%oUfL7C-tZkQcH3y&pVgM8D+u#lRdI4z*qWiN9E0v!nSeJ5A(sXjOaF1x$oTfK6B)tYkPbG2y%pUgL7D- uZlQcI3z&qVhN8E+v#mSdJ4A*rWiO9F0w!nTeK5B(sYjPaG1x%oUfL6C-tZkQbH2y&pVgM7D+u#lRcI4z*qWhN9E0v!mSeJ5A(rXjOaF1w$nTfK6B)sYkPbG2x%pUgL7C-uZlQcH3z&qVhM8D+v#mRdI4A*rWiN9F0w!nSeK5B(sXjPaG1x$oUfL6C)tYkQbH2y%pVgM7D-u#lRcI3z*qWhN8E0v!mSdJ5A(rXiO9F1
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