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Chronic inflammatory demyelinating polyneuropathy


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Question Question


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My father was diagnosed with CIDP last year at age 75. I am curious about the genetic factors involved and if it is a hereditary condition. I would appreciate any information on this disease that you can provide.

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The following information may help to address your question:

  • What causes chronic inflammatory demyelinating polyneuropathy (CIDP)?
  • Is chronic inflammatory demyelinating polyneuropathy (CIDP) inherited?
  • How might chronic inflammatory demyelinating polyneuropathy (CIDP) be treated?
  • What is the long-term outlook for people with chronic inflammatory demyelinating polyneuropathy (CIDP)?

What causes chronic inflammatory demyelinating polyneuropathy (CIDP)?

The underlying cause of CIDP is unknown. There is evidence that it is related to the immune system, and that it may have multiple triggers.[1] It is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath of the peripheral nerves.[2] The myelin sheath is the protective covering of nerve fibers.[3] When myelin is damaged or removed, electrical impulses are slowed or lost, and messages transmitted from the brain are disrupted.[4]
Last updated: 2/27/2017

Is chronic inflammatory demyelinating polyneuropathy (CIDP) inherited?

CIDP is not known to be inherited and is considered an acquired disorder. No clear genetic predisposition or other predisposing factors for CIDP have been identified.[1]
Last updated: 2/27/2017

How might chronic inflammatory demyelinating polyneuropathy (CIDP) be treated?

Standard treatment options for CIDP include:[3][4][5]
  • intravenous immune globulin (IVIG) - adds large numbers of antibodies to the blood plasma to reduce the effect of the antibodies that are causing the problem
  • glucocorticoids - help reduce inflammation and relieve symptoms
  • plasma exchange - removes harmful antibodies from the blood
The choice of treatment may depend on the preference of the patient, side effects, treatment cost, duration, and availability.[5] There are advantages and disadvantages of each treatment option:[5]
  • IVIG and plasma exchange may lead to a more rapid improvement in CIDP than glucocorticoid therapy, but are less likely than glucocorticoids to produce a remission
  • IVIG is expensive, and its supply is sometimes limited
  • Glucocorticoids are inexpensive, but chronic use is limited by common and important side effects
  • Plasma exchange is expensive, invasive, and available only at specialized centers
Other drugs may be used when standard treatments fail or cause significant side-effects.[4] Physical therapy may improve muscle strength, function and mobility.[2]
Last updated: 2/27/2017

What is the long-term outlook for people with chronic inflammatory demyelinating polyneuropathy (CIDP)?

The long-term outlook (prognosis) for people with CIDP appears to vary, but data regarding the prognosis is limited.[2][5] Post-treatment life can depend on whether the disease was caught early enough to benefit from treatment options. For example, gradual onset of CIDP can delay diagnosis by several months or even years, resulting in significant nerve damage.[4]

Complete remission, partial remission, and severe disability have all been reported.[5] Some people may have a "bout" of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses. Some people are left with residual numbness or weakness that can lead to reduced quality of life and/or long-term care.[2][4][6] Rare, severe outcomes that have been reported include quadriplegia, respiratory failure and death.[6]
Last updated: 2/27/2017

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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References References


  1. Richard A Lewis. Chronic inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis. UpToDate. Waltham, MA: UpToDate; January, 2017; http://www.uptodate.com/contents/chronic-inflammatory-demyelinating-polyneuropathy-etiology-clinical-features-and-diagnosis.
  2. NINDS Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Information Page. NINDS. November 6, 2015; https://www.ninds.nih.gov/Disorders/All-Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy-CIDP-Information-Page.
  3. Chronic inflammatory polyneuropathy. MedlinePlus. May 30, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/000777.htm.
  4. Recently Diagnosed with CIDP. GBS/CIDP Foundation International. 2017; https://www.gbs-cidp.org/cidp/all-about-cidp/.
  5. Richard A Lewis. Chronic inflammatory demyelinating polyneuropathy: Treatment and prognosis. UpToDate. Waltham, MA: UpToDate; October, 2015; http://www.uptodate.com/contents/chronic-inflammatory-demyelinating-polyneuropathy-etiology-clinical-features-and-diagnosis.
  6. Jean-Michel Vallat. Chronic inflammatory demyelinating polyneuropathy. Orphanet. December, 2010; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2932.

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