Rare Nephrology News

Disease Profile

Chronic inflammatory demyelinating polyneuropathy

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

1-9 / 100 000

US Estimated

Europe Estimated

Age of onset

Adolescent

ICD-10

G61.8

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

CIDP; Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Summary

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]

Symptoms

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.

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
Do you have updated information on this disease? We want to hear from you.

Cause

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]

Diagnosis

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]

Treatment

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]

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

Organizations

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

      Learn more

      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.

          References

          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; https://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; https://www.uptodate.com/contents/chronic-inflammatory-demyelinating-polyneuropathy-etiology-clinical-features-and-diagnosis.
          8. Jean-Michel Vallat. Chronic inflammatory demyelinating polyneuropathy. Orphanet. December, 2010; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2932.

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