Frequency and time to relapse after discontinuing 6-month therapy with IVIg or pulsed methylprednisolone in CIDP

Nobile‐Orazio, Eduardo; Cocito, Dario; Jann, Stefano; Uncini, Antonino; Messina, Paolo; Antonini, Giovanni; Fazio, Raffaella; Gallia, Francesca; Schenone, Angelo; Francia, Ada; Pareyson, Davide; Santoro, Lucio; Tamburin, Stefano; Cavaletti, Guido; Giannini, F.; Sabatelli, Mario; Beghi, Ettore

doi:10.1136/jnnp-2013-307515

Cited by 73 publications

(61 citation statements)

References 23 publications

(27 reference statements)

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“…In 1 series, 25% of patients remained in remission after a mean follow‐up of 4 years . Further recently published data have similarly suggested remission rates of about 20% . Considerably higher remission rates have also been reported, ranging from 30% to 47% .…”

Section: Ivig For Cidp: For How Long?mentioning

confidence: 85%

“…Despite the lack of evidence of greater efficacy in severe disease, it may be argued that IVIg, in view of the latest trial data, represents a more justified first choice if function is severely impaired, because it offers a higher likelihood of side‐effect‐free therapeutic response in the short term. However, in cases with relatively mild functional disability, not urgently requiring rapid improvement and without contraindication to corticosteroids, the latter may need to be considered as first line, given the possible greater chance of future relapse‐free treatment withdrawal, or a longer period of remission . IVIg is an appropriate second‐line option in these patients if the steroid response is inadequate or if side‐effects occur.…”

Section: Some Proposals For Clinical Practicementioning

confidence: 99%

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Long‐term Immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy

Rajabally

2015

Muscle and Nerve

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Immunoglobulins are an effective but expensive treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Although the goal is to improve function, use of functional scales to monitor therapy is not widespread. Limited recent evidence suggests that doses lower than those used traditionally may be as effective. There are no proven correlations of effective dose with weight, disease severity, or duration. The clinical course of CIDP is heterogeneous and includes monophasic forms and complete remissions. Careful monitoring of immunoglobulin use is necessary to avoid overtreatment. Definitive evidence for immunoglobulin superiority over steroids is lacking. Although latest trial evidence favors immunoglobulins over steroids, the latter may result in higher remission rates and longer remission periods. This article addresses the appropriateness of first-line, high-dose immunoglobulin treatment for CIDP and reviews important clinical questions regarding the need for long-term therapy protocols, adequate monitoring, treatment withdrawal, and consideration of corticosteroids as an alternative to immunoglobulin therapy.

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Section: Ivig For Cidp: For How Long?mentioning

confidence: 85%

Section: Some Proposals For Clinical Practicementioning

confidence: 99%

Long‐term Immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy

Rajabally

2015

Muscle and Nerve

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“…Upon withdrawal of the maintenance treatment, the relapse rate is high: 45% with IVIg and 50% with oral corticosteroids in the 6 months following interruption. In a recent trial, comparing IVIg to methylprednisolone, a similarly high proportion of relapses after discontinuation (87%) was found in the two groups, even if the median time to relapse was significantly longer after methylprednisolone than after IVIg . Consequently, around 40% to 65% of patients require a maintenance treatment and become dependent on the treatment in the long term .…”

Section: Introductionmentioning

confidence: 92%

Rituximab in chronic inflammatory demyelinating polyradiculoneuropathy with associated diseases

Roux

Debs

Maisonobe

et al. 2018

J Peripheral Nervous Sys

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We aimed to analyse the response to rituximab in a cohort of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients with associated disorders. We conducted a clinical and electrophysiological retrospective monocentric study in 28 CIDP patients. Response to rituximab was defined as (a) a five-point increase in the Medical Research Council sum score or a one-point decrease in the Overall Neuropathy Limitations Scale score, compared to the score at the first rituximab infusion, or (b) the discontinuation of, or reduced need for, the last treatments before rituximab initiation. Twenty-one patients (75%) were responders to rituximab. The median time before response was 6 months (1-10 months). Only two patients needed to be treated again during a median follow-up of 2.0 years (0.75-9 years). Interestingly, the response rate was good in patients with associated autoimmune disease (5/8) and similar to the response rate observed in patients with a haematological disease (16/20) (P = 0.63). A shorter disease duration was associated with a better clinical response to rituximab (odds ratio 0.81, P = 0.025) and the response rate was better (P = 0.05) in common forms (83.3%) than in sensory forms (42.9%). No major adverse events were recorded. Rituximab is efficacious in CIDP patients with haematological or autoimmune disease. It improves clinical response and decreases dependence on first-line treatments.

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“…After 12 months, 54% of IVIG‐treated subjects did not require further therapy. Long‐term follow‐up of IVIG‐treated subjects revealed that approximately 86% would worsen within a median follow‐up time of 42 months (range 1–57 months) after therapy discontinuation (median time to worsening 4.5 months, range 1–24 months) …”

Section: Discussionmentioning

confidence: 99%

Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: Results from the pre‐randomization phase of the Polyneuropathy And Treatment with Hizentra study

Mielke¹,

Bril²,

Cornblath³

et al. 2019

J Peripheral Nervous Sys

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In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), intravenous immunoglobulin (IVIG) is recommended to be periodically reduced to assess the need for ongoing therapy. However, little is known about the effectiveness of restabilization with IVIG in patients who worsen after IVIG withdrawal. In the Polyneuropathy And Treatment with Hizentra (PATH) study, the pre‐randomization period included sudden stopping of IVIG followed by 12 weeks of observation. Those deteriorating were then restabilized with IVIG. Of 245 subjects who stopped IVIG, 28 did not show signs of clinical deterioration within 12 weeks. Two hundred and seven received IVIG restabilization with an induction dose of 2 g/kg bodyweight (bw) IgPro10 (Privigen, CSL Behring, King of Prussia, Pennsylvania) and maintenance doses of 1 g/kg bw every 3 weeks for up to 13 weeks. Signs of clinical improvement were seen in almost all (n = 188; 91%) subjects. During IVIG restabilization, 35 subjects either did not show CIDP stability (n = 21, analyzed as n = 22 as an additional subject was randomized in error) or withdrew for other reasons (n = 14). Of the 22 subjects who did not achieve clinical stability, follow‐up information in 16 subjects after an additional 4 weeks was obtained. Nine subjects were reported to have improved, leaving a maximum of 27 subjects (13%) who either showed no signs of clinical improvement during the restabilization phase and 4 weeks post‐study or withdrew for other reasons. In conclusion, sudden IVIG withdrawal was effective in detecting ongoing immunoglobulin G dependency with a small risk for subjects not returning to their baseline 17 weeks after withdrawal.

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Frequency and time to relapse after discontinuing 6-month therapy with IVIg or pulsed methylprednisolone in CIDP

Cited by 73 publications

References 23 publications

Long‐term Immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy

Long‐term Immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy

Rituximab in chronic inflammatory demyelinating polyradiculoneuropathy with associated diseases

Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: Results from the pre‐randomization phase of the Polyneuropathy And Treatment with Hizentra study

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