National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Chronic inflammatory demyelinating polyneuropathy


Other Names:
CIDP; Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Subtypes:
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder that causes progressive weakness and impaired sensory function in the legs and arms. Symptoms often include tingling or numbness (first in the toes and fingers), weakness of the arms and legs, loss of deep tendon reflexes, fatigue, and abnormal sensations.[1] Other symptoms may include pain, difficulty swallowing (dysphagia), and double vision (diplopia).[2] CIDP is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath (protective cover of nerve fibers) of the peripheral nerves.[3] CIDP is closely related to Guillain-Barre syndrome (GBS) and is considered the "chronic counterpart" of GBS. Treatment may include corticosteroids, immunosuppressant drugs, plasma exchange, physical therapy, and/or intravenous immunoglobulin (IVIG) therapy.[1] Left untreated, 30% of people with CIDP will progress to wheelchair dependence.[4]
Last updated: 2/27/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Areflexia
Absent tendon reflexes
0001284
Fatiguable weakness of proximal limb muscles 0030200
Motor conduction block 0012078
Paresthesia
Pins and needles feeling
Tingling
[ more ] 		0003401
Segmental peripheral demyelination/remyelination 0003481
Sensory ataxia 0010871
Unsteady gait
Unsteady walk
0002317
30%-79% of people have these symptoms
Difficulty climbing stairs
Difficulty walking up stairs
0003551
Difficulty walking
Difficulty in walking
0002355
Falls 0002527
Hand muscle weakness 0030237
5%-29% of people have these symptoms
Spontaneous pain sensation 0010833
Percent of people who have these symptoms is not available through HPO
Acute demyelinating polyneuropathy 0007131
Autosomal dominant inheritance 0000006
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Last updated: 7/1/2020
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.[5] It is thought to be caused by the immune system mistakenly attacking and damaging the myelin sheath of the peripheral nerves.[1] 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.[6]
Last updated: 2/27/2017
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.[5]
Last updated: 2/27/2017
The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) should be considered in people with symmetric or asymmetric polyneuropathy who have progressive or relapsing-remitting symptoms for more than two months - particularly if the symptoms include positive sensory symptoms (such as tingling), proximal weakness, or absent reflexes.[5]

The initial diagnosis of CIDP is based on signs and symptoms, but the diagnosis can be confirmed by evidence of peripheral nerve demyelination. This may be identified by either electrodiagnostic testing or by nerve biopsy. Electrodiagnostic testing is recommended for all patients with suspected CIDP.[5] There is general agreement among the medical community that the following criteria support the diagnosis of "classic" CIDP:
  • Progression over at least two months
  • Weakness more than sensory symptoms
  • Symmetric involvement of arms and legs (the same symptoms on both sides)
  • Proximal muscles (those closer to the trunk) involved along with distal muscles (those further from the trunk)
  • Reduced deep tendon reflexes throughout (found by the clinician "tapping" the knee, outside of the elbows, crooks of the arms, wrists and ankles)
  • Increased cerebrospinal fluid protein without pleocytosis (an increased cell count)
  • Nerve conduction evidence of a demyelinating neuropathy
  • Nerve biopsy evidence of segmental demyelination (degeneration of the myelin sheath with sparing of the axon) with or without inflammation[5]
Additional studies that may be indicated include MRI, evaluation for inherited neuropathies, and various laboratory tests. There are no laboratory test findings that specifically point to CIDP, but they may be useful to look for other disorders that have similar symptoms.[5]

A treatment trial may be indicated if the diagnosis remains unclear despite a thorough evaluation. A positive response to immunotherapy may add supportive evidence to the diagnosis.[5]
Last updated: 2/27/2017
Standard treatment options for CIDP include:[3][6][7]
  • 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.[7] There are advantages and disadvantages of each treatment option:[7]
  • 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.[6] Physical therapy may improve muscle strength, function and mobility.[1]
Last updated: 2/27/2017

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified (Brand name: Gammaked) - Manufactured by Kedrion Biopharma
    FDA-approved indication: The treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) to improve neuromuscular disability and impairment and for maintenance therapy to prevent relapse, primary humoral immunodeficiency (PI) in patients 2 years of age and older, and idiopathic thrombocytopenic purpura (ITP) to raise platelet counts to prevent bleeding or to allow a patient with ITP to undergo surgery.
    National Library of Medicine Drug Information Portal
  • Immune globulin injection [human], 10% caprylate/chromatography purified (Brand name: Gamunex-C) - Manufactured by Grifols
    FDA-approved indication: Treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) to improve neuromuscular disability and impairment and for maintenance therapy to prevent relapse. Also for use for the treatment of primary immunodeficiency disease (PIDD) in patients 2 years of age and older and idiopathic thrombocytopenic purpura (ITP).
    National Library of Medicine Drug Information Portal
  • Immune Globulin Subcutaneous (Human), 20% Liquid (Brand name: Hizentra) - Manufactured by CSL Behring
    FDA-approved indication: March 2018 approved for the treatment of adult patients with chronic inflammatory demyelinating polyneuropathy (CIPD) as maintenance therapy to prevent relapse of neuromuscular disability and impairment.
    National Library of Medicine Drug Information Portal
  • Immune globulin intravenous (human), 10% liquid (Brand name: Privigen) - Manufactured by CSL Behring
    FDA-approved indication: September 2017, immune globulin intravenous (human), 10% liquid (Privigen) was approved for the treatment of adults with chronic inflammatory demyelinating polyneuropathy (CIDP) to improve neuromuscular disability and impairment, however it was not studied for use longer than 6 months. It was also approved for the treatment of patients age 15 years and older with chronic immune thrombocytopenic purpura (ITP) to raise platelet counts, and as replacement therapy for primary humoral immunodeficiency (PI).
    National Library of Medicine Drug Information Portal
The long-term outlook (prognosis) for people with CIDP appears to vary, but data regarding the prognosis is limited.[1][7] 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.[6]

Complete remission, partial remission, and severe disability have all been reported.[7] 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.[1][6][8] Rare, severe outcomes that have been reported include quadriplegia, respiratory failure and death.[8]
Last updated: 2/27/2017

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include chronic acquired polyneuropathies (monoclonal gammopathies, diabetes, toxic neuropathies) or inherited neuropathies (Charcot-Marie-Tooth disease or transthyretin amyloid neuropathy; see these terms).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Chronic inflammatory demyelinating polyneuropathy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Chronic inflammatory demyelinating polyneuropathy by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

  • Patient Services, Inc (PSI) provides financial support and guidance for qualified patients with specific chronic diseases. PSI helps patients find solutions to the social and economic problems confronting patients with chronic conditions.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The GBS/CIDP Foundation International provides information about this condition. The GBS|CIDP Foundation International is a global non-profit organization supporting individuals and families affected by Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), and related syndromes through a commitment to support, education, research, and advocacy.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Chronic inflammatory demyelinating polyneuropathy. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • What are the relapse rates for people recovering from this condition? Also what are the percentages of permanent disability related to CIDP? I am currently receiving treatment and recovering but worried about the chances of relapse and the possibility of permanent disability. Thank you for your answers. See answer

  • 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. See answer

  • My friend was diagnosed with chronic inflammatory demyelinating polyneuropathy several months ago. He has been treated with platelet transfusions and gabapentin without relief. Are there other options for treatment? What is the outlook for this condition? See answer


  1. 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.
  2. Chronic Inflammatory Demyelinating Polyneuropathy. National Organization for Rare Disorders (NORD). 2015; https://rarediseases.org/rare-diseases/chronic-inflammatory-demyelinating-polyneuropathy/.
  3. Chronic inflammatory polyneuropathy. MedlinePlus. May 30, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/000777.htm.
  4. What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?. GBS/CIDP Foundation International. 2017; https://www.gbs-cidp.org/cidp/.
  5. 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.
  6. Recently Diagnosed with CIDP. GBS/CIDP Foundation International. 2017; https://www.gbs-cidp.org/cidp/all-about-cidp/.
  7. 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.
  8. Jean-Michel Vallat. Chronic inflammatory demyelinating polyneuropathy. Orphanet. December, 2010; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2932.