Treatment guidelines recommend IVIg or SCIg for the treatment of CIDP. Although IVIg is frequently used as induction or maintenance therapy, it can be associated with several side effects for some patients which include venous thrombosis, hemolytic anemia, aseptic meningitis, and renal complications. IVIg is administered in-clinic and requires 3-5 hours per session. SCIg should be used for maintenance therapy and is associated with frequent minor side effects (mainly skin reactions).8,9
Patient portrayal
CIDP can be debilitating to patients’ lives2
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, often progressive, immune-mediated neuromuscular disorder of the peripheral nervous system.1,3
CIDP results in motor and sensory impairment in the upper and lower limbs.1
CIDP significantly impacts quality of life.1,2
Patients experience a range of disabling mobility and sensory issues.1
Common motor and sensory symptoms include:
IVIg, corticosteroids, and PLEX are effective therapies and guideline-recommended treatments for CIDP8
Both short- and long-term effectiveness, risks, ease of implementation and cost should be considered during treatment decision-making8
Treatment options for CIDP include:8
Observational studies and clinical practice experience suggest that corticosteroids are effective in managing the symptoms of CIDP. In some cases, long-term use of steroids for CIDP can be associated with potential side effects including weight gain, hyperglycemia, hypertension, osteoporosis, cataracts, and increased risk for infection.8
In some patients with good vascular access, plasma exchange (PLEX) may be an acceptable option for chronic treatment. PLEX is associated with some limitations as it requires good vascular access and specialized equipment and is often used after IVIg/SCIg and corticosteroids for this reason.8
Non-steroidal immunosuppressive therapies (NSISTs, e.g., azathioprine, ciclosporin, mycophenolate mofetil) are used as add-on medication or after failure of IVIg/SCIg and corticosteroids. Azathioprine and mycophenolate mofetil are frequently used in CIDP as immunoglobulin- or corticosteroid-sparing agents, although their effectiveness to lower immunoglobulin or corticosteroid dose is uncertain.8
Orthoses, physiotherapy, occupational therapy, psychological support and referral to a rehabilitation specialist can also be considered based on patient needs.8
Existing treatments, while beneficial, may be associated with limitations
CDAS=CIDP Disease Activity Status; CIDP=chronic inflammatory demyelinating polyneuropathy; EFNS=European Federation of Neurological Societies; IVIg=intravenous immunoglobulin; PLEX=plasma exchange; PNS=Peripheral Nerve Society; SCIg=subcutaneous immunoglobulin.
* Remission (CDAS=2) defined as stable and off treatment for <5 years. Seventeen patients (15%) achieved cured disease activity status (CDAS=1), defined as stable and off treatment for ≥5 years.10,11
† A cross-sectional study of 112 patients in the Netherlands with a confirmed CIDP diagnosis from patient files and expert consensus using the 2010 EFNS/PNS criteria. At study entry, median age was 62 years, median disease duration was 9 years, and median time from onset to diagnosis was 5 months (2-12 months). Eighty percent of patients presented with typical CIDP, and 20% presented with CIDP variants.10
‡ Residual neurological symptoms included muscle weakness (73%), sensory symptoms (77%), pain (52%), and fatigue (77%).10
References: 1. Bunschoten C et al. Lancet Neurol. 2019;18:784-94. 2. Querol L et al. J Neurol. 2021;268(10):3706-16. 3. Brun S et al. Immuno. 2022;2:118-31. 4. Mendoza M et al. Adv Ther. 2023;40(12):5188-203. 5. Dyck PJB et al. Mayo Clin Proc. 2018;93(6):777-93. 6. Michaelides A et al. Pain Ther. 2019;8(2):177-85. 7. Gable KL et al. Muscle Nerve. 2020;1-8. 8. Van den Bergh PYK et al. Eur J Neurol. 2021;28:3556-83. 9. Canadian Blood Services. Transfusion – Chapter 4: Immunoglobulin Products (2024). 10. Bunschoten C et al. J Peripher Nerv Syst. 2019;24:253-59. 11. Gorson KC et al. J Peripher Nerv Syst. 2010;15:326-33.
