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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
CIDP treatment includes corticosteroids, intravenous immunoglobulin (IVIG), and/or plasma exchange.2
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
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 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
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Medication Resources
[Immune Globulin Infusion 10% (Human) with recombinant Human Hyaluronidase] Solution
[Immune Globulin Infusion(Human)]
Videos
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.
Additional Resources
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).