Skip to main content
Image of syringe and pipets)

Therapeutic Areas and Products

Neurology

Our world-leading immunoglobulin (Ig) franchise includes an intravenous and a subcutaneous option and is the cornerstone of the neurology therapeutic area. Our efforts in this area focus on bringing trusted products and technologies to serve patients with a rare and serious neurologic disease, Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).

About CIDP

What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)​?
  • CIDP is a neurological disorder characterized by progressive weakness and impaired sensory function, including loss of reflexes.
  • CIDP is considered an autoimmune disorder, in which the immune system begins to attack the peripheral nerves, although the exact mechanisms by which this happens are still not clearly defined.
  • The antigenic target is unknown in the majority of CIDP, and no reliable biologic markers have been identified, adding to the complexity of managing this disorder.
  • The incidence of CIDP is approximately 0.33 per 100,000 individuals per year and prevalence is approximately 2.81 per 100,000 individuals however may be as high as 9 per 100,000.
  • CIDP most commonly occurs between ages 40 and 60 and is more common in men than in women.
  • CIDP is one of a spectrum of acquired demyelinating neuropathies that differ in their time course and clinical features. CIDP has a similar presentation to that of Guillain-Barre Syndrome (GBS), except that GBS is acute and usually peaks within 4 weeks, whereas CIDP is chronic (8 weeks or more) and may be progressive or relapsing.
    • Part of the difficulty in CIDP is getting an accurate diagnosis due to many variants of CIDP as there are both typical and atypical forms. The existence of several phenotypes within CIDP suggests that it may be a spectrum of related conditions rather than a discrete disease.
What are the symptoms of CIDP?​

The classic clinical symptoms of CIDP include:

  • progressive, symmetric weakness of the arms and legs, especially in proximal muscles
  • loss of sensation (numbness); abnormal sensations (tingling, buzzing)
  • progressive or relapsing loss of reflexes (areflexia)
  • unsteady gait (usually resulting from sensory loss and weakness)

Diagnostic tests typically find:

  • electrophysiological features of demyelination (e.g., reduced conduction velocities)
  • high protein levels in spinal fluid analysis
  • inflammation and demyelination observed in nerve biopsy samples

The symptoms of CIDP are caused by damage to the myelin sheath of peripheral nerves, but the particular nerves affected can vary from one patient to another.

The course of CIDP varies widely. Some may have a monophasic course of CIDP followed by recovery, while others may have progressive or relapsing-remitting courses.

How is CIDP treated?​

The primary goals of treatment for CIDP are to:

  • reduce symptoms (weakness, sensory loss, imbalance, pain)
  • improve functional status (reduce disability and handicap)
  • maintain long-term remission, if possible

Standard treatments for CIDP include:

  • Intravenous immunoglobulin (IVIg) therapy at high doses
  • Subcutaneous immunoglobulin 20% is also approved for maintenance regimens in patients stabilized on IVIg
  • Corticosteroids, which are similar to naturally occurring anti-inflammatory hormones made by the body. Corticosteroids often improve strength, can be taken by mouth, and are inexpensive, but side effects can limit long-term use.
  • Plasma exchange (PE) or plasmapheresis (PLEX), which may help remove harmful substances in the plasma.

Despite the proven benefits of IVIg in patients with CIDP, such treatment may be associated with certain limitations and drawbacks, including poor venous access in some patients, treatment-limiting systemic adverse reactions, and the requirement for regular visits to infusion centers or home visits.

Without treatment, about 30% of CIDP patients will progress to wheelchair dependence. Early recognition and proper treatment can prevent loss of nerve function and avoid a significant amount of disability.

References

1.Mathey EK, et al. Chronic Inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psych. 2015;86:973-985. 2.Ohyama K, et al. Muscle Atrophy in Chronic Inflammatory Demyelinating Polyneuropathy: A Computed Tomography Assessment. Eur J Neurol. 2014;21:1002-1010. 3.Querol et al. Autoantibodies in Chronic Inflammatory Neuropathies: Diagnostic and Therapeutic Implications. Nat Rev Neurol. 2017 Sep;13(9):533-547. 4.Broers et al. Incidence and prevalence of chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis. Neuroepidemiology. 2019;52(3-4)161-172. 5.Gorson KC. An update on the management of chronic inflammatory demyelinating polyneuropathy. Ther Adv Neurol Disord. 2012 5(6): 359–373. 6.GBS/CIDP Foundation. www.gbs-cidp.org. Accessed March 17, 2021. 7.Allen JA et al. Neurology. 2015 Aug 11;85(6):498-504. 8.Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat rev Neurol. 2011;7(9):507-517. 9.Robertson EE et al. Treatment of chronic inflammatory demyelinating polyneuropathy. Curr Treat Options Neurol. 2010;12(2):84-89. 10.Farmakidis C et al. Immunosuppressive and immunomodulatory therapies for neuromuscular diseases. Muscle Nerve. 2020;61(1):17-25.). 11.Van den Bergh PYK et al. EFNS/PNS guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the EFSN and PNS. Eur J Neurol. 2010;17:356-363. 12.Salameh JS, et al. Safety and efficacy of subcutaneous immunoglobulin in the treatment of neuromuscular disorders. J Clin Neuromusc Dis. 2016;17:110–19.

For U.S. Healthcare Professionals only

The purpose of this CSL Behring Medical Affairs website is to support Healthcare Professionals with scientific information. This website is also a channel for U.S. Healthcare Professionals to submit questions or connect with CSL Behring U.S. Healthcare Professionals. The information provided is for educational purposes only and is not intended to promote any products. By continuing to use this site you are acknowledging that you are a US Healthcare Professional