Subcutaneous immunoglobulin dose titration to clinical response in inflammatory neuropathy

Kapoor, Mahima; Keh, Ryan; Compton, Laura; Morrow, Sarah A.; Gosal, David; Manji, Hadi; Reilly, Mary M.; Lunn, Michael P.; Lavin, Tim; Carr, Aisling

doi:10.1007/s00415-020-10318-3

Cited by 5 publications

(7 citation statements)

References 22 publications

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“…The degree of limitations in activity in our sample corresponds to previous studies that exclusively used RODS scores [7, 31, 32]. In line with earlier studies, we found that MMN not only affects the upper limb, but also results in significant limitations in activities related to standing and walking, indicating a more widespread loss of muscle function in both upper and lower limbs [3, 33].…”

Section: Discussionsupporting

confidence: 89%

Chronic inflammatory neuropathies and their impact on activities and participation

Wonink

Kruithof

Goedee

et al. 2023

Euro J of Neurology

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Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self-reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies.Aim: To systematically assess the self-reported limitations in activities and participation and determine associated factors. Methods: A survey study was conducted in 2021 in a cohort of patients with CIDP (n = 257) and MMN (n = 148) from a university hospital. The survey included the Raschbuilt Overall Disability Scale and the Utrecht Scale for Evaluation of Rehabilitation-Participation, questions addressing personal and disease-related factors and treatment.Multivariate linear regression analysis was used to determine associations with diseaserelated and personal factors.Results: A total of 147 CIDP and 103 MMN patients responded. Limitations in activities were reported by 70.7% CIDP and 52.2% MMN patients with moderate to severe limitations in 22.4% and 5.9% patients, respectively. Participation restrictions were reported by 50% of CIDP and 40% of MMN patients, nevertheless satisfaction with participation was high. Fatigue, pain and resilience were independently associated with limitations in activities and satisfaction with participation in CIDP patients.Conclusions: Activity limitations and restrictions in participation are common in CIDP patients and to a lesser extent in MMN patients. Fatigue, pain and resilience independently contributed to perceived limitations in CIDP patients. Referral to a rehabilitation physician is warranted to address these limitations appropriately.

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Section: Discussionsupporting

confidence: 89%

Chronic inflammatory neuropathies and their impact on activities and participation

Wonink

Kruithof

Goedee

et al. 2023

Euro J of Neurology

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“…A major challenge remains accurately determining ongoing disease activity in the maintenance phase of regular Ig treatment. In well‐documented patient cohorts, there is marked variation of dosing regimens among patients 4,11‐13 . If the neuropathy is in remission, intermittent treatment reduction or cessation is indicated.…”

Section: Introductionmentioning

confidence: 99%

“…Intravenous (IV) Ig is well-established as an effective treatment, improving strength and function in both CIDP and MMNCB, 1,2 and in recent years subcutaneous (SC) Ig has been recognised as a relevant treatment option with better tolerance, equivalent efficacy and less peri-dose fluctuation compared to IVIg. 3,4 CIDP is clinically and mechanistically a heterogenous disease. In clinical practice, optimal treatment response is achieved by individualised Ig dosing to clinical effect assessed by disease-specific, clinimetrically validated outcome measures.…”

Section: Introductionmentioning

confidence: 99%

“…In well-documented patient cohorts, there is marked variation of dosing regimens among patients. 4,[11][12][13] If the neuropathy is in remission, intermittent treatment reduction or cessation is indicated. Small variations between assessments may represent measurement noise.…”

Section: Introductionmentioning

confidence: 99%

“…In chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMNCB), immunoglobulin (Ig) therapy is the mainstay of treatment for many patients. Intravenous (IV) Ig is well‐established as an effective treatment, improving strength and function in both CIDP and MMNCB, 1,2 and in recent years subcutaneous (SC) Ig has been recognised as a relevant treatment option with better tolerance, equivalent efficacy and less peri‐dose fluctuation compared to IVIg 3,4 …”

Section: Introductionmentioning

confidence: 99%

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Predicting long‐term trends in inflammatory neuropathy outcome measures using latent class modelling

Keh

Selby

Jones

et al. 2022

J Peripheral Nervous Sys

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Immunoglobulin (Ig) is used to treat chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy with conduction block (MMNCB). Regular infusions may be used for symptom control. Disease activity is monitored with clinical outcome measurements. We examined outcome measure variation during clinically stable periods in Ig‐treated CIDP and MMNCB patients. We explored utility of serial outcome measurement in long‐term outcome prediction. Retrospective longitudinal analysis of a single neuroscience centre's Ig‐treated CIDP and MMNCB patients, 2009‐2020, was performed. Mean and percentage change for grip strength, Rasch‐built overall disability scales (RODS) and MRC sum scores (MRC‐SS) during periods of clinical stability were compared to score‐specific minimal clinically important differences (MCID). Latent class mixed modelling (LCMM) was used to identify longitudinal trends and factors influencing long‐term outcome. We identified 85 CIDP and 23 MMNCB patients (1423 datapoints; 5635 treatment‐months). Group‐averaged outcome measures varied little over time. Intra‐individual variation exceeded MCID for RODS in 44.2% CIDP and 16.7% MMNCB datapoints, grip strength in 10.6% (CIDP) and 8.8%/27.2% (MMNCB right/left hand) and MRC‐SS in 43.5% (CIDP) and 20% (MMNCB). Multivariate LCMM identified subclinical trends towards improvement (32 patients) and deterioration (73 patients) in both cohorts. At baseline, CIDP ‘deteriorators’ were older than ‘improvers’ (66.2 vs 57 years, P = .025). No other individual factors predicted categorisation. The best model for ‘deteriorator’ identification was contiguous sub‐MCID decline in more than one outcome measure (CIDP: sensitivity 74%, specificity 59%; MMNCB: sensitivity 73%, specificity 88%). Outcome measure interpretation determines therapeutic decision‐making in Ig‐dependent neuropathy patients, but intra‐individual variation is common, often exceeding MCID. Here we show sub‐MCID contiguous changes in more than one outcome measurement are a better predictor of long‐term outcome.

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Subcutaneous immunoglobulins (SCIG) for chronic inflammatory demyelinating polyneuropathy (CIDP): A comprehensive systematic review of clinical studies and meta-analysis

Ramzi,

Maya,

Balousha

et al. 2024

Neurol Sci

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Background Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presents significant treatment challenges due to its chronic nature, varied clinical presentations, and rarity. Subcutaneous immunoglobulin (SCIG) has emerged as a maintenance therapy, offering potential advantages in administration and patient experience over the previously recognized intravenous immunoglobulin (IVIG). Methods: We included all clinical studies involving CIDP patients treated with SCIG from eleven databases up to March 2024. Results 50 clinical studies were included in the systematic review, with 22 involved in the meta-analysis. These studies offer clinical data on around 1400 CIDP patients. Almost all studies considered SCIG a maintenance therapy, with the majority of results suggesting it as a viable substitute that may offer comparable or enhanced advantages. Studies covered aspects such as efficacy, safety, quality of life, practicality, economic evaluation, and patient preference. Meta-analysis showed SCIG significantly improved muscle strength and sensory function, had fewer and milder side effects, reduced relapse rates, and received a strong preference. Conclusions Findings suggest that SCIG for CIDP maintenance not only provides a more feasible alternative, with economic evaluations showing considerable cost reductions over time, and patient preference for SCIG being pronounced, but may also deliver comparable or superior health outcomes. Ongoing research lines on formulations, techniques, and direct comparative studies are critical to further illuminate, enhance, and expand SCIG's role in treatment.

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Subcutaneous immunoglobulin dose titration to clinical response in inflammatory neuropathy

Cited by 5 publications

References 22 publications

Chronic inflammatory neuropathies and their impact on activities and participation

Chronic inflammatory neuropathies and their impact on activities and participation

Predicting long‐term trends in inflammatory neuropathy outcome measures using latent class modelling

Subcutaneous immunoglobulins (SCIG) for chronic inflammatory demyelinating polyneuropathy (CIDP): A comprehensive systematic review of clinical studies and meta-analysis

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