Standardized Tapering off Subcutaneous Immunoglobulin in Chronic Inflammatory Demyelinating Polyneuropathy.

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY Journal of neuromuscular diseases Pub Date : 2023-01-01 DOI:10.3233/JND-221615
Lars K Markvardsen, Søren H Sindrup, Ingelise Christiansen, Aisha M Sheikh, Jakob V Holbech, Henning Andersen
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引用次数: 1

Abstract

Background: Attempting discontinuation of treatment in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) is recommended. However, there is no evidence based regimen for tapering off subcutaneous immunoglobulin (SCIG). This trial investigated stepwise tapering off SCIG to detect remission and the lowest effective dosage. During tapering off, frequent vs less frequent clinical evaluation was compared. Methods: Patients with CIDP receiving a stable SCIG dosage followed a standardized tapering off regimen: 90%, 75%, 50%, 25% and 0% of the initial dose every 12th week, pending no deterioration occurred. In case of relapse during tapering off, the lowest effective dose was identified. Treatment with SCIG was registered for two years after participation. Disability score and grip strength were primary parameters. Participants were randomized to clinical evaluation every 6th week (frequent) or 12th week (less frequent). Results: Fifty-five patients were included of which thirty-five relapsed. Twenty patients (36%) were able to discontinue treatment without relapse. In relapsing patients, median dosage could be reduced by 10% (range, 0–75). After two years, 18 of 20 patients were still in remission without treatment. Frequent clinical evaluation did not detect deterioration more frequently than less frequent evaluation; RR 0.5 (95% CI, 0.2–1.2) (p = 0.17). Conclusion: In stable CIDP patients, SCIG could be completely tapered off in 36% of the patients and only in 10% of these patients relapse occurred during the following two years. More frequent evaluation was not superior to detect deterioration.

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慢性炎症性脱髓鞘性多神经病的标准化皮下免疫球蛋白减量。
背景:建议慢性炎症性脱髓鞘性多发性神经病(CIDP)患者尝试停止治疗。然而,没有基于证据的方案可以逐渐减少皮下免疫球蛋白(SCIG)。该试验研究了逐步减少SCIG以检测病情缓解和最低有效剂量。在逐渐减少的过程中,比较了频繁和不频繁的临床评估。方法:接受稳定SCIG剂量的CIDP患者遵循标准的减量方案:每12周初始剂量的90%、75%、50%、25%和0%,直到没有恶化。在逐渐减少期间复发的情况下,确定最低有效剂量。SCIG治疗在参与后注册两年。残疾评分和握力是主要参数。参与者被随机分为每6周(频繁)或第12周(不频繁)进行一次临床评估。结果:55名患者被纳入其中,35名复发。20名患者(36%)能够停止治疗而没有复发。复发患者的中位剂量可减少10%(范围0-75)。两年后,20名患者中有18名未经治疗仍处于缓解状态。频繁的临床评估并没有比不频繁的评估更频繁地发现恶化;RR 0.5(95%CI,0.2-1.2)(p = 0.17)。结论:在稳定的CIDP患者中,36%的患者的SCIG可以完全减弱,只有10%的患者在接下来的两年中复发。更频繁的评估并不优于检测恶化。
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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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