Chronic inflammatory demyelinating polyneuropathy is an immune-mediated polyneuropathy characterized by symmetric weakness of proximal and distal muscles and by progression continuing > 2 months (8 weeks).
Symptoms of chronic inflammatory demyelinating polyneuropathy (CIDP) resemble those of Guillain-Barré syndrome. Progression for > 2 months differentiates CIDP from Guillain-Barré syndrome, which is monophasic and self-limited. Between 2 to 5% of patients initially diagnosed as GBS are reclassified as CIDP based on disease progression (1).
The cause is thought to be autoimmune, resulting in demyelination.
General reference
1. Odaka M, Yuki N, Hirata K. Patients with chronic inflammatory demyelinating polyneuropathy initially diagnosed as Guillain-Barré syndrome. J Neurol. 2003;250(8):913-916. doi:10.1007/s00415-003-1096-y.
Symptoms and Signs of CIDP
CIDP typically starts insidiously and may slowly worsen or follow a pattern of relapses and recovery; between relapses, recovery may be partial or complete. Flaccid weakness, usually in the limbs, predominates in most patients; weakness is typically more prominent than sensory abnormalities (eg, paresthesias of hands and feet). Deep tendon reflexes are lost.
In most patients, autonomic function is affected less than in Guillain-Barré syndrome, Also, weakness may be asymmetric and progress more slowly than in Guillain-Barré syndrome.
Diagnosis of CIDP
Cerebrospinal fluid (CSF) analysis and electrodiagnostic tests
Testing includes CSF analysis and electrodiagnostic tests. Results are similar to those in Guillain-Barré syndrome, including albuminocytologic dissociation (increased protein but normal white blood cell count) and demyelination, detected by electrodiagnostic testing.
Nerve biopsy, which can also detect demyelination, is seldom needed.
Treatment of CIDP
IV immune globulin (IVIG)
Glucocorticoids
Plasma exchange
Monoclonal antibodies
IVIG, although more expensive than glucocorticoids, is often offered first to patients with chronic inflammatory demyelinating polyneuropathy because of the following (1):
It does not have the many adverse effects of long-term glucocorticoid use.
It is easier to administer than plasma exchange.
However, evidence suggests that pulsed glucocorticoids may result in longer remissions and have a lower rate of serious adverse effects than IVIG (2). The reason steroids are beneficial in CIDP and not GBS is poorly understood. In CIDP, pulsed glucocorticoids may be given orally or intravenously in a variety of regimens (3, 4):
Oral prednisolone, daily Oral prednisolone, daily
Pulsed high-dose dexamethasone, orally for 4 days every 4 weeks Pulsed high-dose dexamethasone, orally for 4 days every 4 weeks
IV methylprednisolone, weekly or monthlyIV methylprednisolone, weekly or monthly
Some patients may benefit from a combination of IVIG and glucocorticoids.
Plasma exchange also does not have the long-term adverse effects of glucocorticoids, but is invasive, often requires an indwelling port, and may cause hypotension due to large fluid shifts. Plasma exchange may be offered to patients who do not respond to IVIG or who have severe disease. It is best used as a way to de-escalate severe deterioration rather than as long-term maintenance treatment.
Subcutaneous immunoglobulin (SCIG) may be as effective as IVIG (5).
Monoclonal antibodies, such as FcRn inhibitors, were first approved as a treatment of CIDP in 2024. An open-label trial including 221 patients with CIPD found that two-thirds of patients treated with subcutaneous efgartigimod alfa achieved clinical improvement within a median of 4 weeks; among responders, continuation of efgartigimod reduced the risk of relapse by 61% compared to placebo in the randomized phase of the trial (Monoclonal antibodies, such as FcRn inhibitors, were first approved as a treatment of CIDP in 2024. An open-label trial including 221 patients with CIPD found that two-thirds of patients treated with subcutaneous efgartigimod alfa achieved clinical improvement within a median of 4 weeks; among responders, continuation of efgartigimod reduced the risk of relapse by 61% compared to placebo in the randomized phase of the trial (6). Other immunosuppressants (eg, azathioprine, cyclophosphamide, mycophenolate) may be helpful and can reduce glucocorticoid dependence.). Other immunosuppressants (eg, azathioprine, cyclophosphamide, mycophenolate) may be helpful and can reduce glucocorticoid dependence.
Treatment may be needed for a long time.
Treatment references
1. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 26 (3):242–268, 2021. doi: 10.1111/jns.12455
2. Nobile-Orazio E, Cocito D, Jann S, et al. Frequency and time to relapse after discontinuing 6-month therapy with IVIg or pulsed methylprednisolone in CIDP. . Frequency and time to relapse after discontinuing 6-month therapy with IVIg or pulsed methylprednisolone in CIDP.J Neurol Neurosurg Psychiatry. 86 (7):729–734, 2015. doi: 10.1136/jnnp-2013-30751
3. Fehmi J, Bellanti R, Misbah SA, Bhattacharjee A, Rinaldi S. Treatment of CIDP. Pract Neurol. 2023;23(1):46-53. doi:10.1136/pn-2021-002991
4. Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol. 2019;18(8):784-794. doi:10.1016/S1474-4422(19)30144-9
5. Hadden, RDM, Marreno F. Switch from intravenous to subcutaneous immunoglobulin in CIDP and MMN: Improved tolerability and patient satisfaction. Ther Adv Neurol Disord. 8 (1): 14–19, 2015. doi: 10.1177/1756285614563056
6. Allen JA, Lin J, Basta I, et al. Safety, tolerability, and efficacy of subcutaneous efgartigimod in patients with chronic inflammatory demyelinating polyradiculoneuropathy (ADHERE): a multicentre, randomised-withdrawal, double-blind, placebo-controlled, phase 2 trial. Lancet Neurol. 2024;23(10):1013-1024. doi:10.1016/S1474-4422(24)00309-0
Key Points
Although symptoms of chronic inflammatory demyelinating polyneuropathy resemble those of Guillain-Barré syndrome, the 2 can be differentiated based on how long symptoms have continued to progress (ie, > 2 months for CIDP).
Symptoms start insidiously and may slowly worsen or follow a pattern of relapses and recovery.
CSF analysis and electrodiagnostic test results are similar to those of Guillain-Barré syndrome.
Treat with IVIG and glucocorticoids; in severe cases, consider plasma exchange.
Immunosuppressants may help and can reduce dependence on glucocorticoids.
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