Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy

Markvardsen, Lars Kjøbsted; Debost, Jean-Christophe; Harbo, Thomas; Sindrup, Søren Hein; Andersen, Henning; Christiansen, I; Otto, Marit; Olsen, Niels Kjær; Lassen, Lisbeth; Jakobsen, Johannes

doi:10.1111/ene.12080

Cited by 124 publications

(143 citation statements)

References 27 publications

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“…Toxicity and tolerability have been tested and the use has been approved by the FDA. s.c. IgG has been shown to be safe in individuals with immunological neurological diseases like multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy [8,9,10]. Although the number of the cases is small, this therapy is well tolerated and the reports suggest a good response to s.c. IgG treatment.…”

Section: Discussionmentioning

confidence: 99%

Susac Syndrome Treated with Subcutaneous Immunoglobulin

Kleffner

Dörr²,

Duning³

et al. 2013

Eur Neurol

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Background: Susac syndrome is a rare disease characterized by the triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss mainly affecting young women. The finding of antibodies against the endothelium in the sera of these patients has supported the hypothesis of an autoimmune endotheliopathy of the brain, inner ear and retina. Because of the rarity of the disease, treatment is based on the knowledge of case reports and small case series. Medical therapy consists of glucocorticoids, immunosuppressants, acetyl salicylic acid, and immunomodulatory agents such as intravenous immunoglobulin. Methods: We present the case histories of 2 young women with Susac syndrome presenting with several episodes of encephalopathy, branch retinal artery occlusions, and hearing loss that were treated with different immunosuppressive drugs, glucocorticoids and intravenous immunoglobulin. In the course of the disease, the treatment was successfully switched to subcutaneous immunoglobulin without any further relapse in both patients. Conclusion: We conclude that the application of subcutaneous immunoglobulin is easy to learn, helps to reduce in-hospital costs and enables a more flexible everyday life. The treatment with subcutaneous immunoglobulin helps to reduce immunosuppressants and appears to prevent relapses.

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Section: Discussionmentioning

confidence: 99%

Susac Syndrome Treated with Subcutaneous Immunoglobulin

Kleffner

Dörr²,

Duning³

et al. 2013

Eur Neurol

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“…Isokinetic testing protocols, described by Harbo et al 39 have been used as an outcome measure in studies exploring the safety and efficacy of subcutaneous administration of immumoglobulins in CIDP. [40][41][42] Cost and space constraints limit the utility of isokinetic dynamometry in the routine clinical care setting. The assessment also can be timely to perform and requires expertise on the part of the evaluator to become familiar with the testing protocols.…”

Section: Manual Muscle Strength Testing and Isokinetic Strength Testingmentioning

confidence: 99%

Optimizing the Use of Outcome Measures in Chronic Inflammatory Demyelinating Polyneuropathy

Allen¹,

Gelinas

Lewis

et al. 2017

US Neurology

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The challenges encountered during the assessment of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are many. Ideally, CIDP outcome measures capture impairments in disability, strength, and sensory dysfunction, and quality of life (QoL). A number of outcome measures have been validated for this purpose. Disability outcomes include the adjusted inflammatory neuropathy cause and treatment (INCAT) disability score, INCAT overall disability sum score (ODSS), and overall neuropathy limitations scale (ONLS). A more sensitive disability score, the inflammatory Rasch-built overall disability scale (I-RODS), has also been validated for use in clinical trials and may better capture clinically meaningful changes in those with CIDP. Strength and sensory impairment can be assessed in a number of ways, including the INCAT sensory subscore (ISS), Medical Research Council sum score, and Martin vigorimeter or Jamar dynamometer grip strength. However, the feasibility of applying and interpreting these measures during routine daily practice has been questioned. Furthermore, these outcome measures may not reflect other factors that can impair QoL in those affected by CIDP, such as pain and fatigue. A valid, reliable, and responsive composite measure that addresses all aspects of impairment faced by patients with CIDP remains an unmet need in clinical practice.

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“…The consequence is a higher and more stable IgG level, which may prevent end-of-dose reduction in efficacy and minimize side effects [51]. A double-blind, placebo-controlled RCT of SCIG, performed in 30 patients with CIDP who were previously responders to IVIG, showed that there was no significant difference in Medical Research Council (MRC) sum score or grip dynamometry, but there was a significant improvement in isokinetic muscle strength in SCIG-treated patients [52]. Moreover, 70 % of patients preferred SCIG administration.…”

Section: Ivigmentioning

confidence: 99%

Immunotherapy in Peripheral Neuropathies

Léger¹,

Guimarães‐Costa²,

Muntean³

2016

Neurotherapeutics

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Immunotherapy has been investigated in a small subset of peripheral neuropathies, including an acute one, Guillain-Barré syndrome, and 3 chronic forms: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and neuropathy associated with IgM anti-myelin-associated glycoprotein. Several experimental studies and clinical data are strongly suggestive of an immune-mediated pathogenesis. Either cell-mediated mechanisms or antibody responses to Schwann cell, compact myelin, or nodal antigens are considered to act together in an aberrant immune response to cause damage to peripheral nerves. Immunomodulatory treatments used in these neuropathies aim to act at various steps of this pathogenic process. However, there are many phenotypic variants and, consequently, there is a significant difference in the response to immunotherapy between these neuropathies, as well as a need to improve our knowledge and long-term management of chronic forms.

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Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy

Abstract: SCIG treatment in CIDP is feasible, safe and effective, and seems an attractive alternative to IVIG.

Cited by 124 publications

References 27 publications

Susac Syndrome Treated with Subcutaneous Immunoglobulin

Susac Syndrome Treated with Subcutaneous Immunoglobulin

Optimizing the Use of Outcome Measures in Chronic Inflammatory Demyelinating Polyneuropathy

Immunotherapy in Peripheral Neuropathies

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