Panzyga® for your patients with CIDP

CIDP is a neurological disorder associated with an immune-mediated response directed primarily against the peripheral nervous system.1 CIDP manifests with peripheral nerve demyelination.2 Typical CIDP begins with paraesthesia and weakness in the distal limbs as well as difficulty with walking.2 The clinical examination shows progressive symmetric proximal and distal muscle weakness, sensory loss, and decreased or absent deep tendon reflexes.3 The disease course is steadily progressive for more than 8 weeks, but can be relapsing-remitting.3 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 and they include:3

• 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

CIDP is considered an orphan disease and prevalence rates hugely vary in different geographical regions,2 from 1 to 9 per 100,000 affected people.2 Although the reason for these differences remain unknown, different applied diagnostic criteria could be potential explanations.2 CIDP creates a major burden on patients, from physical to psychosocial aspects, including impaired physical function, pain, fatigue and depression.4 Beyond this, CIDP is associated with significant economic impact due to direct costs of illness and indirect costs of loss of productivity.4

Treatment of patients with CIDP is complex and requires individualised strategies.2 Based on the extensive practical experience of effectiveness, immunomodulation with IVIg or treatment with corticosteroids are effective and strongly recommended by international guidelines for both induction and maintenance treatment.3 Plasma exchange is also an effective treatment option and strongly recommended if IVIg and corticosteroids are ineffective.3

Trial overview5,6

*Patients were screened, and, if eligible, entered the wash-out phase, during which their current medication was reduced in a predefined standard manner, to determine whether they had active CIDP. For those previously on IVIg, the dose was reduced by 25% at each sequential infusion until deterioration. For those previously on corticosteroids, the dose was reduced at the discretion of the investigator at a rate to expect study entry within 6–12 weeks and to a dose of prednisolone ≤20 mg/day or equivalent when deterioration occurred. Low doses of corticosteroids at deterioration could be continued during the study.5,6
†Including patients with MADSAM or pure motor CIDP.5,6

‡ Patients deteriorating after Week 18 or 21 dropped out and had their end of study visit at Week 21 or 24, respectively. Patients in the 2g/kg arm were discontinued from the study if they were stable (unimproved) at Week 6 or deteriorated after Week 3 and before Week 21.5,6

§ A responder was defined as a subject who showed an improvement (decrease) ≥ 1 point in adjusted INCAT score at Week 6 compared with baseline (first visit of the Dose-evaluation Phase), completed the 24-week study, and maintained the response at Week 24.5,6
EFNS/PNS, European Federation of Neurological Societies and Peripheral Nerve Society; INCAT, inflammatory neuropathy cause and treatment (INCAT) disability score; MADSAM, multifocal acquired demyelinating sensory and motor neuropathy.

Efficacy

Proven efficacy as maintenance therapy with high response rates.6

By the end of the study, around 80% of patients receiving panzyga® maintenance treatment at 1.0 g/kg showed an improvement in arm and leg disability of 1 point or more, as measured by adjusted INCAT score.6
Efficacy was supported by the proportion of responders in the 0.5 g/kg and 2.0 g/kg dose arms in the adjusted INCAT disability score. In addition, a high proportion of patients showed improvements in the ability to perform daily activities, as measured by the inflammatory Rasch-built Overall Disability Scale (I-RODS), and in muscle strength as measured by the Medical Research Council (MRC) sum scores and grip strength.6

*The lower CI limit of 69% exceeded the predefined threshold of 42%, thus the primary endpoint was met.6
CI, confidence interval; INCAT, inflammatory neuropathy cause and treatment (INCAT) disability score; I-RODS, inflammatory Rasch-built overall disability scale; MRC, Medical Research Council.

†A responder was defined as a subject who showed an improvement (decrease) ≥ 1 point in adjusted INCAT score at Week 6 compared with baseline (first visit of the Dose-evaluation Phase), completed the 24-week study, and maintained the response at Week 24.6

Tolerability

Safety profile consistent with what expected for IVIg products6

The incidence of related treatment-emergent adverse events (TEAEs) was similar across all groups.6

The only TEAE where a dose effect was apparent was headache, with an incidence of 2.9% in the 0.5 g/kg group, 14.5% in the 1.0 g/kg group, and 23.7% in the 2.0 g/kg group.6
The evaluation of safety and tolerability parameters showed that administration of IVIg in the ProCID study was safe in patients with CIDP. In a largely IVIg-naïve patient population, the profle of TEAEs was as expected for IVIg products. Headache was the only dose-dependent TEAE.7

References

1. Briani C, et al. Neurol Sci 2022;43(Suppl 2):605–614;

2. Lehmann HC, et al. J Neurol Neurosurg Psychiatry 2019;90:981–987;

3. Van den Bergh PYK, et al. J Peripher Nerv Syst 2021;26:242–268;

4. Querol L, et al. J Neurol 2021;268:3706–3716.

5. Cornblath DR, et al. J Peripher Nerv Syst 2018;23:108–114;

6. Cornblath DR, et al. Brain 2022;145:887–896.

7. Cornblath DR, et al. Drug Saf 2023;46:835–845. 

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IMPORTANT: The information on this website is based on the European Summary of Product Characteristics (EU SmPC).
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