11 Alerting Facts About Chronic Inflammatory Demyelinating Polyneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy

On February 14, 2017, the U.S. Food and Drug Administration (FDA) accepted the Biologics License Application for the use of Privigen in treating Chronic Inflammatory Demylenating Polyneuropathy or CIDP.1 Privigen, a 10 percent liquid Intravenous Immune Globulin (IVIG), is currently used to treat primary immunodeficiency and immune thrombocytopenic purpura. However, research has proven that it can also be used as a CIDP treatment. A Phase 3 study, for example, showed that patients who have this condition and were treated with Privigen had a response rate of 60.7 percent.2

This is good news for those who have CIDP. But, if you’ve just been diagnosed with it or if you suspect that you have it, this isn’t the only thing you need to know. Here are some surprising facts that will help you understand this condition:

1.    It’s believed to be an autoimmune disease

Nerve fibers are bundled in groups and covered by the myelin sheath — similar to how electrical wirings are covered with polymer insulation.3 The myelin sheath helps electrical impulses travel quickly along the nervous system and ensure all parts of the body receive messages from the brain and vice versa. In patients with chronic inflammatory demyelinating polyneuropathy, the myelin sheath is damaged and inflamed, which prevents the nerves from functioning properly and causes pain, fatigue, weakness, and numbness.4

Experts are not really sure what causes CIDP but there is strong evidence that it’s an autoimmune disease. This means that even though your myelin sheaths are normal and healthy, your immune system considers them as foreign objects and subsequently attacks them. 3 One study shows that some patients with CIDP have anti-NF155 IgG4 antibodies in their blood.5 This can mean that their immune system is attacking neurofascin, a type of protein that helps promote good nerve conduction. 6

2.    It affects thousands of people

Chronic inflammatory demyelinating polyneuropathy is a rare disease but, if you have been diagnosed with this condition, this doesn’t mean that you’re alone. In the United States, there are around 40,000 people who have CIDP, and the number of new cases every year is approximately one to two people in 100,000.7

3.    It’s more common in men and older people

Studies have found out that the disease is more prevalent in men than in women and that many patients develop CIDP in their 60s and 70s. 7 However, if you’re a woman and are relatively young (or either of the two), this doesn’t mean that you can be complacent. Anyone can get CIDP at any time in their life.

4.    There are several variants of CIDP

In classic chronic inflammatory demyelinating polyneuropathy, the patient experiences symmetrical deterioration in both motor and sensory function. It affects all muscles, whether they’re proximal (near the torso) or distal (on the extremities). 3

However, there are types of CIDP that don’t fall under this definition.

  • Pure motor (or motor dominant) CIDP is characterized by weakness and areflexia (loss of reflexes) but doesn’t result to loss of sensation.8
  • Pure sensory (or sensory predominant) CIDP is characterized by sensory loss along with pain, poor balance, and abnormal gait.9
  • Lewis-Sumner syndrome affects distal muscles, primarily in the hands and arms.10
  • Multifocal Motor Neuropathy produces asymmetric weakness muscle weakness and atrophy.11

5.    It’s similar to Guillain-Barre Syndrome

If you look at the symptoms, it’s easy to confuse CIDP with Guillain-Barre Syndrome (GBS). They are related to each other, but they’re not the same.4 GBS almost always comes after a viral infection12, while CIDP is not linked to any infection or illness. In rare cases, GBS can develop a chronic form and become CIDP.13

6.    It’s easy to ignore the early symptoms

The primary symptoms of chronic inflammatory demylenating polyneuropathy involve symmetric weakness in the hip and shoulder muscles as well as those in the hands and feet.14 Abnormal sensation (such as tingling and numbness) or loss of sensation may also be experienced, along with a feeling of fatigue.

Since these symptoms can be attributed to other causes (like old age or over-exertion), many people don’t easily spot the early signs of CIDP. They only pay attention when the symptoms worsen and become difficult to ignore.

7.    It can be difficult to diagnose

There is no single test that will tell you whether you have CIDP or not. To determine if you have this disease, your doctor will ask what symptoms you have been experiencing and when they started. 4 You must be experiencing symptoms for at least two months before CIDP can be confirmed.

Your physician will also conduct a thorough physical examination to test your reflexes, check for muscle atrophy and twitching, loss of sensation, and other signs. Diagnostic tests — such as electromyography, nerve conduction testing, and lumbar puncture — can be done to confirm that you have CIDP. 14

8.    It can be treated

You might think that there’s no cure for chronic inflammatory demyelinating polyneuropathy, but there are actually ways to treat it or at least reduce the severity of symptoms.

Corticosteroids are often used as a first-line treatment to reduce inflammation, although they are not advisable for long-term therapy because of their potentially serious side effects.15 Plasmapheresis (or plasma exchange) is also recommended. In this procedure, the patient’s blood is taken out and the blood cells are returned to his body without the plasma (which contains the antibodies associated with CIDP). According to studies, patients show significant improvement after going through plasmapheresis, although many of them deteriorate weeks or months after the initial treatment and require another plasma exchange session.15

Those with CIDP often receive Intravenous Immune Globulins (IVIG), which contain human antibodies from healthy volunteers and aim to improve the patient’s immune system. 14 This has been the standard treatment for CIDP for several years, although clinical trials are being done to see if Subcutaneous Immunoglobulins (SVIG) can be used instead of IVIG. 16

9.    Prognosis is different for each patient

It’s impossible to predict how soon a CIDP patient would recover and how the disease would affect him in the future. Some patients fully recover and regain their sensory and motor functions, while others are left with permanent nerve damage and experience residual pain, numbness, and tingling. Generally, though, those who get earlier treatment have significantly better prognosis than those who get treatment in the advanced stages of the disease.

10.       There are several ongoing clinical trials for CIDP

If you want to be at the forefront of CIDP treatments, you can sign up for clinical trials. One such trial is testing the effectiveness of using SCIG in treating the disease (ClinicalTrials.gov Identifier:

NCT02465359) while another is testing how IVIG treatments help patients have better grip strength (ClinicalTrials.gov Identifier: NCT02414490). By signing up for clinical trials, you’ll have access to new drugs or procedures that may prove to be more effective in treating CIDP.

11.       You can get support from various organizations

If you have just been diagnosed with CIDP, it’s easy to think that you’re alone. This is especially true if the symptoms bother you too much and your family and friends don’t understand the pain and discomfort you feel. Fortunately, you can seek the help of organizations that offer support to those who are suffering from CIDP. Some examples are the American Autoimmune Related Diseases Association, Inc. and the GBS-CIDP Foundation.

Final Thoughts

Getting diagnosed with chronic inflammatory demylenating polyneuropathy is not easy. But, with the right treatment and support, you can manage the symptoms and enjoy a good quality of life.

 

References:

  1. CSL Behring (2017) FDA Accepts CSL Behring’s Biologics License Application Supplement for Using Privigen® to Treat Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), a Rare Neurological Condition. Available at: http://www.prnewswire.com/news-releases/fda-accepts-csl-behrings-biologics-license-application-supplement-for-using-privigen-to-treat-chronic-inflammatory-demyelinating-polyneuropathy-cidp-a-rare-neurological-condition-300406941.html (Accessed: 18 February 2017).
  1. RareDiseaseReport (2017) FDA Accepts Application for Privigen to Treat CIDP. Available at: http://www.raredr.com/news/fda-accepts-application-for-privigen-to-treat-cidp (Accessed: 18 February 2017).
  1. GBS/CIDP Foundation International (2017) Recently Diagnosed with CIDP. Available at: https://www.gbs-cidp.org/cidp/all-about-cidp/ (Accessed: 18 February 2017).
  1. WebMD (2016) What is chronic inflammatory Demyelinating Polyneuropathy (CIDP)? Available at: http://www.webmd.com/brain/what-is-cidp#1 (Accessed: 18 February 2017).
  1. Devaux, J.J., Miura, Y., Fukami, Y., Inoue, T., Manso, C., Belghazi, M., Sekiguchi, K., Kokubun, N., Ichikawa, H., Wong, A.H.Y. and Yuki, N. (2016) ‘Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy’, Neurology, 86(9), pp. 800–807. doi: 10.1212/wnl.0000000000002418.
  1. Zhang, A., Desmazieres, A., Zonta, B., Melrose, S., Campbell, G., Mahad, D., Li, Q., Sherman, D.L., Reynolds, R. and Brophy, P.J. (2015) ‘Neurofascin 140 Is an Embryonic Neuronal Neurofascin Isoform That Promotes the Assembly of the Node of Ranvier’, Journal of Neuroscience, 35(5), pp. 2246–2254. doi: 10.1523/jneurosci.3552-14.2015.
  1. American Association of Neuromuscular & Electrodiagnostic Medicine (no date) Chronic Inflammatory Demyelinating Polyneuropathy. Available at: http://www.aanem.org/Patients/Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy (Accessed: 18 February 2017).
  1. Kimura, A., Koumura, A., Sakurai, T., Hayashi, Y. and Inuzuka, T. (2013) ‘Pure Motor Chronic Inflammatory Demyelinating Polyneuropathy (P01.151)’, Neurology, 80(7 Supplement), pp. 1–151.
  1. Mathey, E.K., Park, S.B., Hughes, R.A.C., Pollard, J.D., Armati, P.J., Barnett, M.H., Taylor, B.V., Dyck, P.J.B., Kiernan, M.C. and Lin, C.S.-Y. (2015) ‘Chronic inflammatory demyelinating polyradiculoneuropathy: From pathology to phenotype’, Journal of Neurology, Neurosurgery & Psychiatry, 86(9), pp. 973–985. doi: 10.1136/jnnp-2014-309697.
  1. Genetic and Rare Diseases Information Center (2016) Lewis-Sumner syndrome. Available at: https://rarediseases.info.nih.gov/diseases/13070/lewis-sumner-syndrome (Accessed: 18 February 2017).
  1. Genetic and Rare Diseases Information Center (2016) Multifocal motor neuropathy. Available at: https://rarediseases.info.nih.gov/diseases/11011/multifocal-motor-neuropathy (Accessed: 18 February 2017).
  1. National Institute of Neurological Disorders and Stroke (2011) Guillain-Barré Syndrome Fact Sheet. Available at: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet (Accessed: 18 February 2017).
  1. Johns Hopkins Medicine (2015) Guillain-Barre and CIDP. Available at: http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/peripheral_nerve/conditions/guillain_barre_and_cidp.html (Accessed: 18 February 2017).
  1. National Organization for Rare Disorders, Inc. (no date) Chronic Inflammatory Demyelinating Polyneuropathy. Available at: https://rarediseases.org/rare-diseases/chronic-inflammatory-demyelinating-polyneuropathy/ (Accessed: 18 February 2017).
  1. Gorson, K.C. (2012) ‘An update on the management of chronic inflammatory demyelinating polyneuropathy’, Therapeutic Advances in Neurological Disorders, 5(6), pp. 359–373. doi: 10.1177/1756285612457215.
  1. Hadden, R.D.M. and Marreno, F. (2014) ‘Switch from intravenous to subcutaneous immunoglobulin in CIDP and MMN: Improved tolerability and patient satisfaction’, Therapeutic Advances in Neurological Disorders, 8(1), pp. 14–19. doi: 10.1177/1756285614563056.

 

 

 

 

 

 

 

 

 

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