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Not an actual patient.
The pathophysiology of CIDP is thought to be based on the autoimmune destruction of peripheral nerve myelin,2 leading to characteristic clinical symptoms of progressive limb weakness with impaired sensory function.2,3 Diagnosis of CIDP is challenging, and laboratory features, electrodiagnostic and clinical criteria are key.4
The age of CIDP onset is usually 40 to 60 years, although it can occur at any age. CIDP is twice as common in men than women.5 Available evidence suggests that ethnicity does not influence rates of CIDP.6
Several lines of evidence support the hypothesis that CIDP is caused by autoimmune-mediated destruction of peripheral nerve myelin. Probable target antigens are adhesion molecules in both non-compact myelin, as well as regions where Schwann cells and axons interact.2,7,8 Although no principal pathogenic autoantibody or triggering antigen has been identified for the initiation of CIDP immunopathogenesis,2 macrophages are primarily responsible for Schwann cell demyelination, with biopsies also showing nerve infiltrations by CD8+ and CD4+ T cells.2,8 Ongoing systemic inflammation is driven by upregulation of pro-inflammatory Th17 cells and dysfunctional anti-inflammatory Regulation T (Treg) cells.2,8
Two mechanisms are hypothesized to be involved in CIDP immunopathogenesis:
In the first mechanism, antibody binding to nodal proteins directly blocks their function, interfering with saltatory conduction.8 In the second, antibody binding and complement recruitment is followed by deposition of a membrane attack complex (MAC). This leads to Schwann cell lysis and demyelination by macrophages, which disrupts and destroys the node and surrounding areas.8 Antibodies that bind nodal and para-nodal proteins are found more frequently in the serum of patients with CIDP than in controls.8
Challenges exist to diagnosing CIDP,2,8-12 so patients experience median delays of 10 months (range 2-64 months), which could be longer for patients with CIDP variants.9 CIDP diagnosis may be incorrect or delayed due to variations in diagnostic criteria and the existence of stringent electrodiagnostic criteria established for clinical trial screening, which may not capture all patients who could respond to treatment2. Diagnostic criteria for CIDP have been difficult to develop because of disease heterogeneity and variation in disease progression, including non-uniform nerve demyelination, debates on the definition of nerve demyelination, and a lack of diagnostic biomarkers.10
Due in part to the absence of a diagnostic biomarker, and the fact that CIDP is one of the few treatable causes of neuropathy, numerous diagnostic criteria have been developed for CIDP, with the goal of identifying the maximum number of patients for treatment.4,11,13-15
Fifteen formal sets of clinical diagnostic criteria have been published for use in CIDP.11 Four of the most prominent guidelines are from the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS),4 the Inflammatory Neuropathy Cause and Treatment (INCAT) group,13 the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM)16 and the American Academy of Neurology (AAN).17
Although specific diagnostic criteria vary, there is general agreement that clinical and electrophysiologic features are the most important in making an accurate diagnosis.4,11,13-15 Supportive laboratory assessments are used where clinical and electrodiagnostic criteria are not fulfilled.4 Diagnosis is given alongside one of two levels of certainty: CIDP or possible CIDP.4 There is consensus among the different sets of criteria that patients should have reduced or absent reflexes and that the development of motor/sensory dysfunction should manifest over at least 2 months.5,12,14,16 Four of the most prominent sets of criteria differ in terms of electrodiagnostic–test result requirements.4,13,15,17 This may be due to the growing understanding of CIDP as a heterogenous disease.
Updates to the guidelines in 2021 include: the term ‘CIDP variants’ replacing ‘atypical CIDP’, and the number of levels of diagnostic uncertainty decreasing from three (‘definite’, ‘probable’ or ‘possible’) to two (‘definite’ or ‘possible’).4,15 Several neuropathies need to be excluded to make a positive diagnosis of typical CIDP, as listed below:4
CIDP clinical presentation is characterized by insidious onset of symptoms including both proximal and distal progressive, symmetric weakness of the limbs, sensory dysfunction, tingling, paresthesia and pain, and areflexia or diminished tendon reflexes.2,3,19,20 Symptoms are typically ongoing for ≥8 weeks, although disease progression can be monophasic or relapsing-remitting.2,19,20 In some cases, CIDP may be preceded by infection.5
Types of CIDP
Typical CIDP is characterized by motor and sensory symptoms, paresthesia, difficulty walking, progressive, symmetric proximal and distal muscle weakness, sensory loss, decreased and/or absent deep tendon reflexes, and demyelination per EAN/PNS criteria4,7,8
Distal CIDP, also known as distal acquired demyelinating symmetric neuropathy, or DADS, is dominated by sensory symptoms, but motor symptoms may develop4,7,8
Symptoms are typically distal and symmetrical, often associated with abnormally increased distal motor latencies and accentuated weakness in the lower limbs, with gait instability4,7,8
Approximately two thirds of patients have immunoglobulin M (IgM) paraproteinemic neuropathy4
Multifocal CIDP encompasses Lewis-Sumner Syndrome, or LSS, and multifocal acquired demyelinating sensory and motor neuropathy, or MADSAM4
It is characterized by a multifocal distribution of sensory and motor symptoms that usually affect upper limbs first, with later involvement of lower limbs4,8
Cranial nerves are likely to be more frequently involved than in other forms of CIDP, and nerve conduction studies frequently show conduction blocks4,7,8
Patients with Motor CIDP present exclusively with motor deficits, with relatively symmetric proximal and distal weakness and normal sensation4
Nerve conduction may be normal4,7
Patients may deteriorate after corticosteroid treatment4
Sensory CIDP is dominated by sensory symptoms
Gait ataxia and impairment of sensation (vibration, position and cutaneous) are observed, with weakness appearing later in approximately 70% of patients4
Patients may have normal nerve conduction7
This is not intended to be a comprehensive resource of all congresses and congress materials across therapeutic and disease areas. Congress materials may include information about investigational use(s) of compounds/products that are not approved for use by the U.S. Food and Drug Administration (FDA) and/or are inconsistent with the Prescribing Information. Takeda does not recommend the use of any Takeda product beyond the approved labeling. Any decisions regarding the usage of a Takeda product beyond the approved labeling are left to the discretion of the healthcare professional. Takeda makes no representations about whether investigational compounds or unapproved uses will be approved by the FDA.
Global meeting featuring thought leaders from around the world focused on advancing patient care in allergy and immunology.
Premier global educational event for allergists and immunologists, with thousands of attendees each year, discussing allergies, asthma, and immune deficiency disorders.
Each year, the American Academy of Neurology's Annual Meeting offers a robust lineup of diverse learning opportunities covering nearly every topic and subspecialty, helping you stay up to date on the latest trusted science and essential education.
Brings together home and alternate site infusion professionals for four days of networking, education, and exhibits. The expo features companies displaying the latest products and services supporting the industry.
Annual meeting dedicated to the advancement of neuromuscular, musculoskeletal, and electrodiagnostic medicine.
Annual event with more than 2,500 members and non-members of the AMCP to engage on the latest innovations and most intentional networking in managed care pharmacy.
National conference of IgNS bringing together professionals and practitioners from all disciplines and clinical specialties to advance Ig therapy practice, while providing networking and comprehensive education opportunities.
This event brings together over 1200 HAE community members, including families, caregivers, healthcare professionals, and pharmaceutical representatives.
Regional annual conference discussing the most current information from top experts in allergy, asthma, and immunology.
International conference showcasing the latest advances in pulmonary disease, critical illness, and sleep disorders for scientists and clinicians at all stages of their careers.
Annual meeting with the goal of providing a stimulating forum with presentations and discussions on the latest advances in clinical immunology, including primary immunodeficiencies and immune dysregulatory diseases.
Annual meeting in neurology promoting high quality patient-centric care in a variety of topics and specialties through various learning formats.
Large assembly of pharmacy and healthcare professionals dedicated to the issues of managed care pharmacy, offering immersive education sessions and keynote presentations.
Annual conference ideal for infusion professionals or those seeking opportunities in the industry to come together and explore the latest trends in offering home-based services.
Premier global educational event for allergists and immunologists, with thousands of attendees each year, discussing allergies, asthma, and immune deficiency disorders.
Annual scientific session uniting allergists, immunologists, nurses, and physician assistants aimed at maintaining the highest standard of practice in allergy care.
This resource provides information on Takeda medications available in the Chronic Inflammatory Demyelinating Polyneuropathy category and is not intended to represent a complete list of therapeutic options.
[Immune Globulin Infusion (Human)] 10%
[Immune Globulin Infusion (Human), 10% with Recombinant Human Hyaluronidase]
Watch videos focused on Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).
In this video, Chris describes his experience of being diagnosed and living with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).
Julie shares her experience of being diagnosed with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and the challenges of living with this disorder.
Find materials to help foster a deeper understanding of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).
An overview of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) clinical presentation and diagnostic considerations.
An overview of the task force guideline recommendations for diagnosing Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).
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