关键词: chronic inflammatory demyelinating polyneuropathy outcome response timing treatment

来  源:   DOI:10.1111/ene.16399

Abstract:
OBJECTIVE: The amplitude, timing, and determinants of improvement with available treatments are uncertain in chronic inflammatory demyelinating polyneuropathy (CIDP). Our primary objective was to quantify categorized outcomes with routine care.
METHODS: We retrospectively studied treatment response within 36 months from initiation in 112 consecutive subjects with CIDP. Response was classified into a proposed new \"CIDP treatment-response category\" (CT-RC), based on achieved endpoints. Determinants of the CT-RC, of timing of maximum improvement, and of treatment discontinuation were ascertained.
RESULTS: The CT-RC demonstrated high concurrent validity with current outcome measures. Thirty-six subjects (32.1%) achieved a \"complete response,\" 37 (33%) a \"good partial response,\" 10 (8.9%) a \"moderate partial response,\" and 15 (13.4%) a \"poor partial response.\" Fourteen subjects (12.5%) were \"nonresponsive.\" The CT-RC was independently predicted only by age. Mean time to maximum improvement was 12.1 months (range = 1-36) and was not associated with any pretreatment covariate. Treatment discontinuation occurred in 24 of 62 (38.2%) partial responders and was only associated with shorter pretreatment disease duration. Nonresponders were older and received a similar number of treatments compared to responders.
CONCLUSIONS: CT-RC classification indicates persistent disability in >60% of treatment responders in CIDP. Timing of maximum improvement is variable, frequently delayed, and unpredictable. Treatment withdrawal without deterioration is achievable in approximately 40% of subjects and may be more likely with prompt treatment. Treatment withdrawal in partial responders and limited escalation in nonresponders suggest implication of physician- and patient-related factors in suboptimal response. More effective treatments/treatment methods and better understanding of other factors influencing response are needed in CIDP.
摘要:
目标:振幅,定时,在慢性炎症性脱髓鞘性多发性神经病(CIDP)中,现有治疗改善的决定因素尚不确定.我们的主要目标是量化常规护理的分类结果。
方法:我们回顾性研究了112名连续的CI-DP患者在开始治疗后36个月内的治疗反应。反应被归类为拟议的新的“CIDP治疗反应类别”(CT-RC),基于已实现的端点。CT-RC的决定因素,最大改善的时机,并确定治疗中断。
结果:CT-RC显示出与当前结果指标高度并行的有效性。36名受试者(32.1%)达到“完全反应”,“37(33%)a”良好的部分反应,“10(8.9%)a”中度部分反应,“和15(13.4%)a”部分反应不佳。“14名受试者(12.5%)”无反应。“CT-RC仅按年龄独立预测。达到最大改善的平均时间为12.1个月(范围=1-36),与任何治疗前协变量无关。在62名部分反应者中,有24名(38.2%)发生了治疗中止,并且仅与较短的治疗前疾病持续时间有关。与响应者相比,无响应者年龄较大,接受的治疗数量相似。
结论:CT-RC分类显示CIDP>60%的治疗应答者持续残疾。最大改善的时间是可变的,经常延迟,和不可预测的。在大约40%的受试者中可以实现不恶化的治疗退出,并且可能更有可能进行及时治疗。部分反应者的治疗退出和无反应者的有限升级表明,在次优反应中,与医生和患者相关的因素。CIDP需要更有效的治疗/治疗方法和更好地了解影响反应的其他因素。
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