文摘
客观的描述安全、耐受性和应对TPE SPSD患者。
背景僵硬的人综合征谱系障碍(SPSD)是一种罕见的群禁用神经免疫疾病。SPSD往往需要免疫治疗特别是在症状治疗反应不佳的设置。血浆置换治疗的安全性和有效性(TPE)在SPSD尚不清楚。
设计/方法患者病历的回顾性研究SPSD看到约翰霍普金斯医院进行。病人特点、考试结果,诊断研究、治疗反应和TPE-related并发症都被记录下来。我们也进行了线性回归模型来评估可能的预测因素TPE的回应。文献综述SPSD和TPE也进行了。
结果36 SPSD患者接受TPE;平均年龄为48岁,81%为女性,平均anti-GAD65抗体效价是42352 U /毫升(范围0 - 309902)。22患者典型的SPS, 10 SPS-plus, 3有其他的表型。33例患者治疗急性发作,3维护TPE。有4例(11.1%)TPE-related并发症(导管感染、导管血栓形成、出血),但没有死亡或过敏反应。20例(55.6%)报告后症状改善TPE, 13报告没有变化,和3报道症状的恶化。36总病人TPE, 21日收到TPE约翰霍普金斯医院急性恶化的条件下,以12要求少anti-spasmodic药物治疗3个月后TPE治疗。没有积极的治疗反应TPE的预测因素。文献回顾发现42更多的病人;69%的患者报告暂时改善他们的身体状况。
结论我们描述患者的安全性和耐受性TPE SPSD和显示TPE-related并发症并不常见和可控的。此外,许多SPSD派生改善患者TPE。进一步的研究可以帮助临床医生在SPSD何时使用TPE。
脚注
披露:罗伊博士没有披露。Mercure-Corriveau博士没有披露。丹尼尔Obando没有披露。王博士没有披露。道尔博士没有披露。Tobian博士没有披露。布洛赫博士已经收到个人薪酬在500 - 4999美元的范围为作秀的旅级战斗队担任顾问。布洛赫博士已经收到个人薪酬在500 - 4999美元的范围为雅培公司担任顾问。Newsome博士已经收到个人薪酬在500 - 4999美元的范围作为高速公路的顾问。Newsome博士已经收到个人薪酬在500 - 4999美元的范围为在科学咨询服务或数据安全监测委员会生原体。 Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Greenwich Biosciences. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Newsome has received research support from Biogen. The institution of Dr. Newsome has received research support from Genentech/Roche. The institution of Dr. Newsome has received research support from Department of Defense. The institution of Dr. Newsome has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Newsome has received research support from National MS Society. Dr. Newsome has received personal compensation in the range of $5,000-$9,999 for serving as a Clinical adjudication committee member for clinical trial with medDay Pharmaceuticals. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving as a Lead PI for Clinical Trial with Roche.
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