Chronic Inflammatory Demyelinating Polyneuropathy

Who gets Chronic Inflammatory Demyelinating Polyneuropathy?

People of any age and either sex can get CIDP. However, the peak period patients develop CIDP is in the 5th or 7th decade of life and the disorder is twice as common in men. There does not seem to be a genetic link to CIDP and there is no know clear trigger. The incidence of CIDP each year is estimated to be between 1.5 and 3.6 million in the population. Approximately 30,000 patients in the US are affected at any one time.

How is Chronic Inflammatory Demyelinating Polyneuropathy diagnosed?

Diagnosis of CIDP is based on the symptoms of the patient:

Symptoms such as loss of sensation (numbness), abnormal sensation (tingling and pain), loss of reflexes, and weakness (difficulty walking, foot drop) that progress beyond 8 weeks in duration. This is the most common type of CIDP. There are also variants.

Tests such as nerve conduction and EMG (show a  demyelinating neuropathy), spinal fluid analysis (usually showing elevated protein with normal cell count), blood and urine tests (to rule out other disorders that may cause neuropathy and to look for unusual proteins.) It is recommend to reference the electrodiagnostic criteria is the EAN/PNS 2021 guidelines in order to ensure the diagnosis is correct.

How is Chronic Inflammatory Demyelinating Polyneuropathy treated?

Many people with CIDP benefit from  treatment.  Although there is a wide spectrum of how severely people with CIDP can be affected, some with the condition require assistance to perform daily tasks. In the more severe cases devices like wheelchairs or walkers may be needed for mobility. One of the goals of early diagnosis and proper treatment is to avoid this level of disability and to improve the long-term outlook. Risks and benefits of treatment options should be discussed with a physician on an individual basis to find the best option.

Treatments available for CIDP:

• Corticosteroids (Prednisone, Prednisolone) are similar to naturally occurring anti-inflammatory hormones made by the body, and can be used as an initial treatment. Corticosteroids often improve strength, are conveniently taken by mouth, and are inexpensive. Si
• Intravenous Immune Globulins (IVIG)  IVIG contains naturally occurring antibodies obtained from healthy volunteers. IVIG is given through a vein over the course of several hours. There are also newer treatments available with preparations of immunoglobulin that can be given under the skin (subcutaneous) now available for CIDP patients. 

See “Understanding a Self-Infused CIDP Therapy Option” for more information

• Plasma Exchange (PE), or Plasmapheresis (PLEX), is a process by which some of the patient’s blood is removed and the blood cells returned without the liquid plasma portion of the patient’s blood. It may work by removing harmful antibodies contained in the plasma.
• Efgartigimod is the first and only plasma-free treatment approved recently by the FDA for CIDP, and works differently than other CIDP treatments. Efgartigimod can be self injected subcutaneously once weekly by a health care professional or self-administration. 

Per the EAN/PNS guidelines, there are some recommended medications for second-line use for refractory CIDP. These are used in combination or as monotherapy when the above standard treatments fail, cause significant side-effects, or the clinical response is not optimal.  These drugs are largely not tested in randomized controlled trials, but their use is supported by case series from the medical literature

Post-treatment life can be improved if the disease is diagnosed and treated in a timely manner. Patients respond in various ways. One third of patients can achieve medication free remission. However, the chronic nature of CIDP requires long-term care of patients and long term treatment for two -thirds of patients with the diagnosis. Accommodations in the home may be needed to facilitate daily living activities.

Video - https://youtu.be/WJVVYk1sg0I?si=eNeTqCQe5XFs3JbK 

The GBS CIDP Foundation International has publications for patients and their caregivers related to CIDP including:

• An of Overview for the Layperson
• Guidelines for Physical and Occupational Therapy
• A Handbook for Caregivers
• Caring For A Child with GBS or CIDP 

In addition, the GBS CIDP Foundation International provides information about chapter meetings and connecting to local liaisons for support.

See also the AANEM endorsed AAN Guideline on IVIg in Neuromuscular Disorders 

More Information

GBS CIDP Foundation International 
Foundation for Peripheral Neuropathy
American Chronic Pain Association
National Institute of Neurological Disorders and Stroke
Multifocal Motor Neuropathy Page