结节病专题_培训课件.ppt

对于复发和激素抵抗的患者处理原则 细胞毒药物： MTX、CTX、AZT、环孢A、苯丁酸氮芥 非细胞毒药物： 氯喹、己酮可可碱 、反应停 特异性抗TNF-α药物 甲氨蝶呤 MTX 多用于对激素抵抗或复发的慢性结节病 对肺结节病的有效率40-47% 大多病人的激素用量可以减少，副作用少，是一个理想的替代激素的药物 硫唑嘌呤 仅有少量治疗肺结节病的非随机对照研究：可在一定程度上改善病情或减少激素的剂量 报道有限，一般用于严重、难治性结节病，如神经系统病变 环磷酰胺 CTX 被用在难治性结节病 环孢菌素A 最常用于神经系统结节病 缺乏有效依据、价格高、副作用大，不作为常规选择 氯喹，羟氯喹 总有效率低于50%，多被用于疗效比较好判断的病变，如皮肤结节病和高钙血症，其中治疗皮肤结节病的有效率为35% 己酮可可碱 少量个案报道，均取得了比较好的效果 但目前还缺乏关于治疗慢性病例或远期预后的研究 沙利度胺 治疗皮肤病变疗效均比较确切，但对肺结节病只有极少量报道 显著的副作用限制了它的广泛研究和使用 特异性抗TNF-α药物 TNF-α是参与结节病炎症反应的众多细胞因子中最关键的一个 BALF中TNF-α的水平与疾病活动性相平行，并能提示预后 Infliximab（英福利美） 是一种IgG1鼠-人嵌合性TNF-α的单克隆抗体 已有很多治疗成功的病例报道 价格昂贵，还未能广泛应用 Summary of Toxicity and Therapeutic Usage for Steroid Alternatives in Sarcoidosis R. Baughman, M. Judson, U. Costabel, R. Du Bois, M. Drent, M. Kavuru, K. Lo, C. Andresen, R. Schlenker-Herceg, E. Barnathan on behalf of the T48 investigators * * * * * * * * * * Results (DLCO and Cst in non-restrictives): Results (DLCO and Cst in non-restrictives): Results (FEV1/VC and MMEF in all patients): Airflow limitation (lowered FEV1/VC and/or MMEF in all patients): Airflow limitation (lowered FEV1/VC and/or MMEF in ever and never smokers): █ MMEF only, █ MMEF and FEV1/VC, █ FEV1/VC only, █ no airflow limitation Airflow limitation (lowered FEV1/VC and/or MMEF in all patients and acc. to smoking status): 19% 28% 23% Airflow limitation (lowered FEV1/VC and/or MMEF in all patients and acc. to stage): █ MMEF only, █ MMEF and FEV1/VC, █ FEV1/VC only, █ no airflow limitation 17% 31% 23% 33% Restrictive pattern expressed as lowered lung volumes indices was observed only in 7.2% of patients Lung parenchyma involvement pronounced as diminished Cst and/or DLCO was detected in 42% of non-restrictive patients Airflow limitation (reduced FEV1/VC and/or MMEF) was found in approximately 23% of patients regardless of they were smokers or not In 42% of cases lung function indices were in normal range Results: Summary smokers: ever