Chronic inflammatory demyelinating polyneuropathy (CIDP)
An overview of CIDP
What is CIDP?
CIDP (chronic inflammatory demyelinating polyneuropathy) is a chronic and acquired immune-mediated disorder affecting the peripheral nervous system.11,12
If left untreated, CIDP can lead to progressive motor and sensory dysfunction and permanent disability.11,12
Diagnosis
Diagnosis of CIDP can be challenging - the disorder may be present in about 20% of undiagnosed neuropathies,11 and in about 10% of all patients who are referred to neuromuscular clinics.11 Estimates of prevalence vary, but CIDP could affect two to five individuals out of every 100,000 people.11
There are a wide range of presentations seen among people with CIDP.13 Typically, CIPD presents with both sensory and motor nerve dysfunction. Although a sensory-predominant type can also be seen, upon examination these individuals are often revealed to have motor nerve abnormalities.13
CIDP is slightly more prevalent among men than women and is most commonly seen in adults between 40 and 60 years of age, although it can also affect older patients and children.11
Pathophysiology
The mechanisms behind CIDP aren’t fully understood.11 The disorder is characterised by inflammation that damages the protective myelin sheath covering nerve fibres.12,13 This sheath is crucial for normal nerve conduction.13 In some cases, the nerves themselves may be damaged.13
Cellular and humoral immune systems may act together in causing this peripheral nerve damage.13 The underlying immune pathology varies between different people, explaining the clinical variation seen between different patients.13
Some studies suggest that an inflammatory response of autoreactive T cells to peripheral myelin antigens may underlie the pathology.13
Treatment
The good news is that there are treatments available.12 In fact, CIDP is the most common treatable chronic neuropathy worldwide.13 Octagam®, an intravenous immunoglobulin therapy, is one of these treatments.3,4,14
Diagnosis of CIDP
When a patient presents with symptoms that suggest CIDP, multiple approaches may be needed to confirm the diagnosis.12 If the signs and symptoms listed below are seen, there is a strong possibility the patient may have CIDP.12 Diagnosis can be challenging as symptoms vary between patients.13
Expert opinion “Electro-diagnostic testing is crucial to confirm a CIDP diagnosis. MRI with contrast enhancement can reveal inflammation of the nerves in the spinal roots, brachial or lumbar plexus, or peripheral nerves. Nerve ultrasound can also be useful, as it can show nonuniform enlargement of peripheral nerves. A thorough patient history and laboratory examination is mandatory to exclude other possible causes of polyneuropathy, such as diabetes, vitamin deficiency or alcohol abuse. Family history may reveal a hereditary sensory-motor.
Treatment of CIDP
The European Federation of Neurological Societies (EFNS) recommends IVIg therapy as a first-line treatment for all forms of CIDP.12 Octagam® is an IVIg that is approved for patients with CIDP.3,4
The flow chart below illustrates the EFNS recommendations for treating CIDP, based on individual patient symptoms.12
Expert opinion: “IVIg therapy is considered to be more beneficial than corticosteroids for patients who have moderate to severe motor symptoms. As the side effects of corticosteroids can also be avoided if IVIg therapy is used, IVIg should be considered in patients with clear contraindications to steroids, such as unstable diabetes. A ‘watch and wait’ approach may be appropriate for patients."
Treatment of CIDP
The table below explains the EFNS recommendations related to each treatment option.
Expert opinion: “Two to three courses of IVIg treatment might be needed before a benefit is seen. If a benefit is seen, taper the dosage incrementally until the patient is receiving the lowest effective maintenance dose. If there is a relapse with the last dosage, a full course of IVIg (2 g/kg b.w.) should be given, followed by the last effective dosage. The tapering strategy may differ between different treatment centers."
Octagam® benefit for CIDP patients
In an observational, retrospective clinical trial, over 90% of patients with CIDP who were for tolerability treated with octagam®5% either improved (41.7%; 10 of 24 patients), or remained clinically stable (50.0%; 12 of 24 patients).14
Measurement of efficacy
Improvement was defined as a decrease of ≥1 score in the Overall Neuropathy Limitation Scale* in the first 3–6 months after beginning treatment with octagam®5%.14
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).14
* 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.14
Tolerability in CIDP Patients
Octagam® has an established record of proven tolerability across multiple indications.1,2
Our experience in CIDP reinforces this record.14
Tolerability of octagam®5% in a multicentre observational study14
In a multicentre observational study, patients with CIDP* tolerated octagam® well, with a very low rate of adverse drug reactions reported.4 Most patients (41/47 patients) did not report an adverse drug reaction.14
The type, severity and frequency of adverse drug reactions seen in this study were comparable with rates seen in a study of over 6000 patients treated with octagam® who had conditions such as primary and secondary immunodeficiency.2,14
* Patients were adults previously diagnosed with CIDP, for whom octagam®5% treatment had been initiated since 1999 (inclusive).14 Eleven centres contributed to the study data.14 On average, patients were treated for 265.6 days.4 The mean number of treatment courses per patient was 5.2.14 Patients received a mean total dose of 1.3 g/kg/month.14
†Ten adverse drug reactions were experienced by six patients.14
References
1. Frenzel W, et al. Tolerability and safety of octagam® (IVIG): a post-authorization safety analysis of four non-interventional phase IV trials. Int J Clin Pharmacol Ther. 2016;54(11):847-855.
2. Debes A, et al. Tolerability and safety of the intravenous immunoglobulin octagam®: a 10-year prospective observational study. Pharmacoepidemiol Drug Saf. 2007;16(9):1038-1047.
3. octagam®5%. Summary of Product Characteristics. May 2021.
4. octagam®10%. Summary of Product Characteristics. May 2021.
11. Gorson KC. An update on the management of chronic inflammatory demyelinating polyneuropathy. Ther Adv Neurol Disord. 2012;5(6):359-373.
12. Van den Bergh PY, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010;17(3):356-363.
13. Mathey EK, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86(9):973-985.
14. Belmokhtar C, et al. Efficacy and Safety of Octagam in Patients With Chronic Inflammatory Demyelinating Polyneuropathy. Neurol Ther https://doi.org/10.1007/s40120-019-0132-5, 2019.
15. https://emedicine.medscape.com/article/1174021-overview#a5. Sasa Zivkovic, Michael C Isfort, Multifocal Motor Neuropathy with Conduction Blocks. Updated: Nov 12, 2018.
16. Lawson VH and Arnold DW. Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment. Neuropsychiatric Disease and Treatment 2014:10 567-576.
17. Dimachkie MM, et al. Multifocal motor neuropathy, multifocal aquired demyelinating sensory and motor neuropathy, and other chronic aquired demyelinating polyneuropahty variants. Neurol Clin. 2013 May;31(2):533-555.
18. Beadon K, et al. Multifocal motor neuropathy. Curr Opin Neurol 2018, 31:559–564.
19. Cats EA, et al. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology. 2010 Aug 31. 75(9):818-825.
20. Van Schaik I.N. and Joint Task Force of the EFNS and the PNS. EFNS/PNS MMN GUIDELINE. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of multifocal motor neuropathy. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision. Jl of the Peripheral Nervous System 15:295–301 (2010).
21. Vucic S, et al. Multifocal motor neuropathy: decrease in conduction blocks and reinnervation with long-term IVIg. Neurology. 2004 Oct 12. 63(7):1264-1269.
22. Van den Berg-Vos RM, et al. Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment. Brain. 2002 Aug. 125(Pt 8):1875-1886.
23. Hahn A, et al. A controlled trial of intravenous immunoglobulin in multifocal motor neuropathy. J Peripher Nerv Syst. 2013;18:321-330.
24. Franques J, et al. Efficacy and tolerance of octagam® in multifocal motor neuropathy with conduction block (MMN). Poster presented at the INC Rotterdam 2012.
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