Chronic inflammatory Demyelinating Polyneuropathy (CIDP)

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CIDP is a neurological disorder associated with an immune-mediated response directed primarily against the peripheral nervous system.1-3 CIDP manifests with peripheral nerve demyelination.1-3 Typical CIDP begins with paraesthesia and weakness in the distal limbs as well as difficulty with walking.2,4 The clinical examination shows progressive symmetric proximal and distal muscle weakness, sensory loss, and decreased or absent deep tendon reflexes.4 The disease course is steadily progressive for more than 8 weeks, but can be relapsing-remitting.4 However, the disease can also manifest with different phenotypes; these are usually identified as CIDP variants because they share the common features of demyelination and response to immune therapy.4

Diagnostic criteria for CIDP

Clinical criteria⁴

Typical CIDP

All the following4:

Progressive or relapsing, symmetric, proximal and distal muscle weakness of upper and lower limbs, and sensory involvement of at least two limbs
Developing over at least 8 weeks
Absent or reduced tendon reflexes in all limbs

CIDP variants

One of the following, but otherwise as per typical CIDP4

(tendon reflexes may be normal in unaffected limbs):

Distal CIDP: distal sensory loss and muscle weakness predominantly in lower limbs
Multifocal CIDP: sensory loss and muscle weakness in a multifocal pattern, usually asymmetric, upper limb predominant, in more than one limb
Focal CIDP: sensory loss and muscle weakness in only one limb
Motor CIDP: motor symptoms and signs without sensory involvement
Sensory CIDP: sensory symptoms and signs without motor involvement

Demonstrated efficacy in CIDP⁵

In an observational, retrospective clinical trial, over 90% of patients with CIDP who were treated with octagam®5% either improved (41.7%; 10 of 24 patients), or remained clinically stable (50.0%; 12 of 24 patients).5

Measurement of efficacy Improvement was defined as a decrease of ≥1 score in the Overall Neuropathy Limitation Scale at 3–5 months after beginning treatment with octagam®5%. The Overall Neuropathy Limitation Scale measures limitations in the everyday activities of the upper and lower limbs. A score of 0 indicates that a patient has no problems with running, walking or climbing stairs. The scale is based on the Overall Disability Sum Score, which was designed to assess the limitations of people with immune-mediated peripheral neuropathies.

Patient details

Patients with CIDP were either naïve to immunoglobulin treatment (11 patients) or had stopped their previous IVIg therapy ≥12 weeks before the trial (13 patients).5

Well tolerated in CIDP⁶

Octagam® has a well-established record of proven tolerability in CIDP, supported by extensive clinical experience.⁶

Clinical experience shows that octagam®5% and octagam®10% are well tolerated in patients with CIDP*.
Only 0.61% of infusions (5/813) were associated with an AE.6


Number of patients with CIDP	58
Number of infusions	813
Infusions per patient, mean	14
Dosage per treatment cycle, mean (range)	0.8 g/kg

*Data from three open label non interventional studies of a Post-Authorization Safety Surveillance program for a subset of patients receiving octagam®5% or 10% for CIDP.

References

Briani C, et al. Neurol Sci 2021; 43(Suppl 2):605–614;
Lehmann HC, et al. J Neurol Neurosurg Psychiatry 2019; 90:981–987;
Querol L, et al. J Neurol 2021;268:3706–3716.
Van den Bergh P, et al. J Peripher Nerv Syst 2021; 26:242–268;
Belmokhtar C, et al. Neurol Ther 2019; 8, 69–78
Wietek S. Neurodegener Dis Manag 2018; 8:227-231.

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