CIDP

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is a rare, chronic immune-mediated peripheral neuropathy in which the immune system attacks the myelin, the protective layer around peripheral nerves.1-4

Characterised by:

  • Progressive weakness, impaired sensory function in the legs and arms, loss of deep tendon reflexes (areflexia), and fatigue1-5
  • CIDP has been associated with impaired wellbeing and presence of mental health conditions – patients may be reliant on help for everyday tasks1-5

 

Induction and maintenance therapy in CIDP patients according to the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines:6
For CIDP patients experiencing disabling symptoms, Intravenous Immunoglobulin (IVIG) or corticosteroids are recommended as first-line treatments. Plasma exchange offers similar efficacy but may be less tolerated or more challenging to administer. Treatment choice should consider patient-specific contraindications and be guided by shared decision-making.


If the response to initial treatment is inadequate or associated with significant side effects, alternative first-line options should be trialed before considering combination therapy. Adding an immunosuppressant or immunomodulatory drug may be considered, but there is no sufficient evidence to recommend any particular drug. Ongoing treatment decisions should take into account disease activity, relapse history, treatment dependence, and the presence of irreversible axonal damage. If the first-line treatment is effective, continuation should be considered until the maximum benefit has been achieved and then the dose should be reduced or the interval increased to find the lowest effective maintenance dose.


For patients responsive to IVIG with active disease, both subcutaneous immunoglobulin (SCIG) and IVIG can be considered as maintenance treatment. When CIDP patients switch from IVIG to SCIG, it is reasonable to start using the same mean dose (1:1) per week. If the treatment effect is insufficient, the dose should be adjusted using reliable outcome measures.


There is some indication that pulsed corticosteroids may be preferable for long-term treatment effectiveness, because of a possible higher rate and longer duration of remission, or when IVIG is unaffordable or unavailable. For maintenance treatment, non-controlled studies indicated that plasma exchange can still be effective if treatment with IVIG or corticosteroids fails. Plasma exchange requires good vascular access and specialized equipment. For single exchanges during long-term maintenance treatment, tunnelled catheters may be used. These drawbacks make plasma exchange, despite its effectiveness and relative safety, a possible option for chronic treatment after corticosteroids and IVIG.

EAN/PNS guideline for induction therapy 6
 

  • Intravenous immunoglobulins
  • Corticosteroids
  • Plasmapheresis if IVIG and corticosteroids are ineffective

EAN/PNS guideline for maintenance therapy 6
 

  • Subcutaneous immunoglobulins
  • Intravenous immunoglobulins
  • Corticosteroids
  • Plasmapheresis if IVIG and corticosteroids are ineffective

What do CIDP patients value most in their treatment?
 

As part of a post-meeting survey following a patient-led meeting for Guillain-Barré Syndrome (GBS)|CIDP Foundation International, participants (N=184) were asked about the most appealing improvements in their CIDP treatment from a list of options they could select multiple choices.6,7

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Abbreviations: 

CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; EAN, European Academy of Neurology; GBS, Guillain-Barré Syndrome; IVIG, intravenous immunoglobulin; PNS, Peripheral Nerve Society; SCIG, subcutaneous immunoglobulin.

References:

  1. Allen JA, et Neurology. 2015;85(6):498-504.
  2. Dalakas MC. Nat Rev Neurol. 2011;7(9):507-17.
  3. National Organization for Rare Disorders (NORD). CIDP Information page. Available at: https://rarediseases.org/rare-diseases/chronic-inflammatory-demyelinating-polyneuropathy/. Accessed June 2025.
  4. Moun Sinai. Chronic inflammatory demyelinating polyneuropathy. Accessed February, 2025. Chronic inflammatory demyelinating polyneuropathy Information | Mount Sinai - New York
  5. Querol L, et a/. J Neurol. 2021;268(10):3706-16
  6. Van den Bergh PYK et al. Eur J Neurol. 2021; 28(11):3556–3583.
  7. GBS-CIDP.org. Independent study. Voice Of The Patient. Chronic Inflammatory Demyelinating Polyneuropathy. Available at: https://www.gbs-cidp.org/wp-content/uploads/2022/08/GBSCIDP-Voice-of-the-Patient-Report_Final.pdf. Accessed June 2025.