Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

About Chronic Inflammatory Demyelinating Polyneuropathy

About Primary Immunodeficiency Diseases

What is CIDP?

Chronic inflammatory demyelinating polyneuropathy, or CIDP, is a rare disorder characterized by gradually increasing sensory loss and weakness associated with loss of reflexes. This is caused by an autoimmune process in which the body’s own immune system doesn’t recognize its nerve fibers and attacks them.  This leads to inflammation of nerve roots and peripheral nerves, as well as the deterioration of the fatty protective covering (myelin sheath) over the nerves, affecting how fast the nerve signals are transmitted and leading to loss of nerve fibers.1,2

Prevalence & Diagnosis

CIDP is considered a rare disease, with the number of new cases per year averaging about 1-2 per 100,000 people, while the prevalence reflecting the accumulation of cases over time is estimated to be 5-9 per 100,000.1 

CIDP is often difficult to diagnose because it is so rare. If a health care provider suspects CIDP, they may perform the following tests to confirm a diagnosis.2,3

  • Electromyogram (EMG) to look for myelin damage in peripheral nerves
  • Spinal fluid analysis can detect elevated levels of certain proteins related to the disease
  • Blood and urine tests to rule out other disorders

Symptoms

The chief symptoms of CIDP are progressive, symmetric weakness of both muscles around the hip and shoulder as well as of the hands and feet. This pattern of weakness is highly suggestive of CIDP.

Other signs and symptoms of CIDP can include:2

Sensory loss

Imbalance

Numbness

Fatigue

Pain

Clumsiness

Difficulty walking

CIDP Treatment Options

About Primary Immunodeficiency Diseases

The primary goals of treatment for CIDP are to reduce symptoms in order to improve functional status and if possible, maintain long-term remission. Initial treatment options include corticosteroids, therapeutic plasma exchange, and immunoglobulin replacement therapy given by intravenous or subcutaneous administration.4

Immunoglobulin Replacement Therapy

The term “immunoglobulin” (Ig) refers to the component of blood plasma that contains antibodies. Ig replacement therapy works in several ways to treat CIDP, including inhibiting the pathogenic antibody effects that cause CIDP.  This results in reduction of the progression of the disease, and improvement in symptoms.4,5

IVIg is given through a vein over the course of several hours. Newer preparations of higher concentrations that can be given subcutaneously have been tested in controlled trials in CIDP patients. This includes subcutaneous immunoglobulin (Hizentra®) which is indicated for the treatment of adult patients with CIDP as maintenance therapy to prevent relapse of neuromuscular disability and impairment.

Learn more about the differences between IVIg and SCIg

References:

  1. GBS/CIDP Foundation. Recently Diagnosed with CIDP. https://www.gbs-cidp.org/cidp/all-about-cidp/ Accessed June 30, 2020
  2. National Organization for Rare Disorders. Chronic Inflammatory Demyelinating Polyneuropathy. RareDiseases.org. Accessed June 30, 2020
  3. Cedars-Sinai. Chronic Inflammatory Demyelinating Polyneuropathy. Cedars-Sinai.org Accessed June 30, 2020
  4. Ryan M et al. Chronic Inflammatory Demyelinating Polyneuropathy: Considerations for Diagnosis, Management, and Population Health. Am J Manag Care. 2018;24:S371-S379
  5. Jacob et al. Current Proposed Mechanisms of Action of Intravenous Immunoglobulins in Inflammatory Neuropathies. Curr Neuropharm. 2009; 7: 337-342

Resources

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Freedom Infusion Systems Patient Guide

Get a quick overview of the Freedom Infusion System, the benefits of SCIg with Freedom and how to prevent adverse effects with the proper ancillary supplies.
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FREEDOM60® SCIg Infusion Mat

Use this tool to help walk you through your SCIg infusions with the FREEDOM60®.
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FreedomEdge® SCIg Infusion Mat

Use this tool to help walk you through your SCIg infusions with the FreedomEdge®.
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GBS|CIDP Foundation International

The GBS|CIDP Foundation International supports individuals and their families affected by Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and related syndromes.
www.gbs-cidp.org

IG Living

Join the immune globulin community with access to IG Living magazine, advocacy groups, expert opinions, and patients with rare diseases.
www.igliving.com