Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder in which the immune system mistakenly attacks the protective covering of peripheral nerves. This damage disrupts nerve signaling and can lead to muscle weakness, numbness, and problems with balance and coordination. CIDP is estimated to affect between 1 and 9 people per 100,000 individuals.
Cause of CIDP
The exact cause of CIDP is not fully understood, but research shows that it is driven by an abnormal immune response. In CIDP, immune cells and antibodies attack the myelin sheath that surrounds and protects peripheral nerves. This process, called demyelination, slows or blocks the electrical signals that nerves use to communicate with muscles and sensory tissues in the limbs and body.
As the body tries to repair damaged nerves in CIDP, the protective myelin sheath is thought to repeatedly break down and undergo repair. Over time, this cycle can cause structural changes in the nerves and can eventually affect the underlying nerve fibers if inflammation persists.
CIDP is considered an acquired autoimmune disorder, meaning that it develops during life rather than being inherited. The triggers that initiate the immune attack in CIDP are not always clear, although infections, immune system disorders, or other medical conditions may play a role.
Symptoms of CIDP
CIDP occurs more often in adults than in children and is more common in men than in women. Symptoms develop gradually over several months, although some people experience a more rapid onset. In some cases, the disease follows a relapsing-remitting course, meaning that symptoms may worsen for a period of time and then partially improve.
Common symptoms of CIDP include:
· Progressive muscle weakness in the arms and legs
· Difficulty walking, climbing stairs, or standing from a seated position
· Reduced or absent reflexes
· Numbness, tingling, or loss of sensation in the hands and feet
· Problems with balance and coordination
Weakness may affect both proximal muscles (e.g., in the hips and shoulders) and distal muscles (e.g., in the hands and feet). Since sensory nerves are also involved, many people experience difficulty sensing vibration or body position, which can lead to an unsteady gait.
CIDP symptoms can vary widely between individuals. Some people develop CIDP variants, which may cause additional symptoms or present with different patterns of muscle weakness and nerve involvement.
For more information about the symptoms of CIDP, as well as discussion of diagnostic and management concerns, an overview can be found here.
Diagnosis of CIDP
Diagnosing CIDP can be challenging because its symptoms overlap with other nerve disorders. Doctors typically suspect CIDP based on a patient’s medical history, neurological examination, and the pattern of progressive weakness and sensory changes.
To confirm the diagnosis, physicians may perform nerve conduction studies and electromyography (EMG). These tests measure how well electrical signals travel along the nerves and can identify the slowed conduction caused by demyelination.
Additional tests may include:
· Blood tests to rule out other causes of neuropathy
· Lumbar puncture to examine cerebrospinal fluid, which often shows elevated protein levels in CIDP
· MRI or ultrasound imaging to evaluate nerve roots or nerve enlargement
· In selected cases, a nerve biopsy to look for signs of demyelination
Current management of CIDP
CIDP is considered a treatable condition. Treatment focuses on modifying the immune response, reducing inflammation, and preventing further nerve damage. Early diagnosis and treatment can help reduce symptoms, improve strength and mobility, and lower the risk of long-term nerve damage.
The main treatment approaches include:
· Immunotherapy: First-line treatments that target the immune system include corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchange (plasmapheresis), which can help reduce inflammation and remove harmful antibodies from the blood.
· Immunosuppressive medications: For individuals who do not respond to first-line therapies, drugs such as azathioprine, cyclophosphamide, cyclosporine, or rituximab may be used to help control the immune response.
· Supportive care: Physical and occupational therapy, pain management, orthotics, and assistive devices can help maintain strength, coordination, and independence.
Several therapies are approved by the U.S. Food and Drug Administration (FDA) to treat CIDP, including:
· IVIG therapies (such as Gammagard Liquid, Panzyga, and Gamunex-C), which help regulate the immune system and improve nerve function.
· Subcutaneous immune globulin therapies (such as HYQVIA), which are given under the skin as ongoing maintenance treatment.
· Efgartigimod alfa and hyaluronidase-qvf (Vyvgart Hytrulo), approved in 2024 as the first neonatal Fc receptor (FcRn)-blocking therapy for CIDP, which works by lowering harmful antibodies involved in the disease.
Evolving research and treatment landscape
Despite availability of treatment options for CIDP, there are still unmet needs for people with this condition. Many current CIDP treatments, such as IVIG, corticosteroids, and plasma exchange, require frequent administration and may not work for every patient. New therapies being tested in clinical trials aim to provide more targeted immune modulation, longer-lasting effects, and improved quality of life for people living with CIDP. Several promising therapies under investigation include:
Complement inhibition therapies
These therapies are designed to block the complement system, a part of the immune response that can damage the myelin sheath surrounding peripheral nerves.
Riliprubart (Sanofi) – This therapy is being tested in two major phase 3 trials. One is evaluating whether riliprubart improves symptoms in patients whose disease does not respond to standard treatments, while the other compares the drug directly with IVIg, one of the standard first-line treatments for CIDP. Both studies are currently recruiting participants.
SAR445088 (Sanofi) – This complement inhibitor was evaluated in a phase 2 proof-of-concept study to assess safety and effectiveness in patients with CIDP receiving or refractory to standard therapy. This study was completed in late 2025.
DNTH103 (Dianthus Therapeutics) – This complement inhibitor is currently in a phase 3 clinical trial (CAPTIVATE) that is recruiting participants. The study recently moved forward after positive early results. This therapy is designed to provide longer-lasting effects with less frequent dosing.
FcRn inhibitors (antibody-lowering therapies)
A growing area of research focuses on therapies known as FcRn inhibitors, such as efgartigimod. These treatments are designed to lower levels of harmful IgG antibodies in the body, which are thought to play a key role in driving autoimmune diseases like CIDP.
Nipocalimab (Janssen) – This therapy is being evaluated in a phase 2/3 clinical trial designed to determine whether it can prevent disease relapse in people with CIDP. The study is currently recruiting participants.
Batoclimab (Immunovant) – This therapy is being studied in a phase 2 trial to assess its safety and effectiveness in adults with active CIDP. The trial is active, but not currently recruiting participants.
B-cell targeting therapies
Another therapeutic strategy under development focuses on B cells, the immune cells responsible for producing antibodies.
Rituximab (Genentech/Roche) – This B-cell–depleting therapy is being studied in a phase 3 trial (ReCIX) located in Amsterdam to determine whether it can induce remission in patients with CIDP, including those who depend on ongoing IVIG therapy. The study is currently recruiting participants.
To learn more about clinical research opportunities in CIDP, visit clinicaltrials.gov and search for the disease name in the condition or disease field.
MDA’s work to further cutting-edge CIDP research
As part of MDA’s broader mission to support people living with neuromuscular diseases, CIDP was recently added to the Muscular Dystrophy Association (MDA) disease portfolio, reflecting its impact and MDA’s commitment to supporting individuals and families affected by this condition. Through investments in research from MDA, partner advocacy groups, and the National Institutes of Health (NIH), efforts to better understand CIDP and develop improved treatments are advancing, offering hope for future breakthroughs.
MDA’s Resource Center provides support, guidance, and resources for patients and families, including information about CIDP, open clinical trials, and other services. Contact the MDA Resource Center at 1-833-ASK-MDA1 or ResourceCenter@mdausa.org.
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