CIDP is a treatable condition that affects the peripheral nervous system. IVIG, plasma exchange, and corticosteroid therapy are the first-line treatment options, but a newer medication for adults has recently received approval.
Chronic inflammatory demyelinating polyneuropathy (CIDP), also known as
Peripheral nerves are those outside the brain and spinal cord. They transmit motor signals, send sensory information, and regulate involuntary functions such as breathing, digestion, and heart rate.
When the myelin sheath breaks down, it negatively affects the ability of electrical signals to move along the nerve. Signals can slow down, weaken, or fail to reach their destination completely. Symptoms such as muscle weakness, numbness, sensory loss, and reflex loss are common symptoms of this process in CIDP.
With effective treatment, a state of remission in CIDP is possible for some people. This article discusses the first-line therapies for CIDP, alternative options, and the latest medication available.
Doctors treat CIDP as an immune-mediated condition. The condition is associated with immune dysfunction linked to nerve-damaging inflammation. It also involves autoantibodies (immune cells) that mistakenly attack the myelin sheath of peripheral nerves.
Medications and therapies for CIDP focus on modifying immune responses to halt or slow demyelination and inflammation, promoting nerve repair and function. Intravenous immunoglobulin (IVIG), corticosteroids, and plasmapheresis are the cornerstones of treatment.
Intravenous immunoglobulin (IVIG) is an
An initial dose of IVIG for CIDP is administered by infusion into a vein
Doctors typically use IVIG alongside corticosteroids, medications that further regulate immune responses and decrease inflammation.
Corticosteroids are drugs that mimic the action of the body’s natural hormones called glucocorticoids. Glucocorticoids play various roles in regulating immune activation and activity.
In CIDP, corticosteroids suppress immune activity, reducing the active immune response against the myelin sheath of peripheral nerves. Corticosteroids also help lower the amount of pro-inflammatory substances resulting from immune dysfunction.
People can take corticosteroids for CIDP
Plasmapheresis, also known as
Plasmapheresis eliminates many nerve-damaging substances from the bloodstream by replacing the plasma affected by immune dysfunction with a substitute.
In CIDP, doctors typically reserve plasmapheresis for severe cases or situations where IVIG and corticosteroids are not effectively managing symptoms.
During this procedure, a portion of a person’s blood is collected from a vein and placed into a special machine that separates the plasma from the blood cells. The machine mixes a sterile plasma substitute with the blood cells before infusing the whole blood back into the body. This process continues until a significant amount of plasma has been replaced.
Plasma exchanges for CIDP generally take place over
Vyvgart Hytrulo is a medication approved by the
Vyvgart Hytrulo is a combination of the drugs efgartigimod alfa and hyaluronidase. It works by reducing pathogenic (disease-causing) immunoglobulin G (IgG) antibodies in the bloodstream. IgG antibodies are natural components of the immune response that help fight infection, but in CIDP, some IgG antibodies mistakenly attack the myelin sheath.
Efgartigimod alfa is a neonatal Fc receptor blocker. It binds to specific receptor sites on immune cells and prevents the body from recycling pathogenic IgG antibodies to keep them in circulation. With the receptor site blocked by efgartigimod alfa, pathogenic IgG antibodies break down instead of being recycled, reducing their levels.
The enzyme hyaluronidase enhances the absorption and distribution of efgartigimod alfa when administered by injection under the skin (subcutaneously).
Currently, a healthcare professional administers this medication weekly.
Approximately
- methotrexate
- cyclosporine
- cyclophosphamide
- rituximab
- mycophenolate
Additionally, supportive therapies can help a person maintain daily activities while living with CIDP. Physical therapy, occupational therapy, lifestyle modifications, and psychosocial supports may help improve overall quality of life.
Doctors usually tailor a treatment plan for CIDP based on a person’s symptoms, disease progression, lifestyle, and preferences. Not every treatment is appropriate for every person living with CIDP.
Discussing current treatment goals with a doctor is a good way to ensure clarity about treatment. It can also help a person understand why medical professionals recommend certain therapies over others. It is always advisable to ask about new and emerging options or clinical trials.
When questions remain about the appropriate course of action even after a thorough discussion, it is OK to seek a second or third professional opinion.
CIDP is an immune-mediated condition that can cause symptoms such as muscle weakness, numbness, and reflex loss. While it can be significantly impairing, effective treatments are available.
Intravenous immunoglobulin, corticosteroids, and plasmapheresis are the first-line treatments for CIDP. They focus on modifying immune responses that promote inflammation and nerve damage. Vyvgart Hytrulo, the latest FDA-approved medication for adult CIDP, also works by modifying the immune system.
In addition to standard therapies, lifestyle changes, physical therapy, occupational therapy, and psychosocial support may help improve a person’s overall quality of life with CIDP.