CIDP Treatment Options: First-Line and Second-Line Therapies

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Treatments

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Treatments
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If you have chronic inflammatory demyelinating polyneuropathy (CIDP), you may have encountered delays and missteps before finally getting diagnosed. Getting the correct diagnosis can be tricky when you have CIDP. The autoimmune condition is rare, different variants can cause a wide range of symptoms, and many features of the disorder are similar to those of Guillain-Barré Syndrome (GBS). (It’s common for CIDP to be confused with GBS until symptoms persist longer than eight weeks, which is when doctors may suspect CIDP.)

Fortunately, once you know CIDP is the culprit of challenging symptoms like sensory problems and weakness, you can begin to focus on treating and managing your chronic condition.

First-Line Treatments for CIDP

Your individual CIDP treatment plan will depend on factors such as symptom severity, nerve damage, and what your doctor thinks will work best to preserve and restore function.

“In CIDP, there are three approved and well-evidenced interventions: plasma exchange, intravenous immunoglobulin, and corticosteroids,” says Michal Vytopil, MD, PhD, vice chair of neurology at Beth Israel Lahey Health’s Lahey Hospital and Medical Center in Burlington, Massachusetts.

Corticosteroids

While these medications can effectively reduce inflammation and improve symptoms in many people with CIDP, they can cause serious side effects such as increased risk of osteoporosis, high blood pressure, and diabetes if used long term. For this reason, they aren’t a viable option for continued use to treat this chronic condition.

Plasma Exchange (Plasmapheresis)

This treatment involves taking blood and using a machine to remove the plasma (the liquid component of blood, which, in people with CIDP, contains harmful antibodies that attack the nerves). It is then replaced with healthy plasma from donors and the blood is put back into the body.

“Plasma exchange is a laborious process, especially if you have to do it chronically,” says Gil Wolfe, MD, SUNY distinguished professor of neurology at Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo. In addition, it generally reduces symptoms for just a few weeks at a time.

Intravenous Immunoglobulin Therapy (IVIG) or Subcutaneous Immunoglobulin Therapy (SCIg)

This treatment involves intravenous (IV) infusions or injections of immunoglobulins, proteins your body makes to protect against invading organisms such as viruses and bacteria.

IVIG or SCIg can help reduce your immune system’s attack on peripheral nerves, but as with plasma exchange, the effects last for only a few weeks, and you’ll need consistent ongoing treatments to manage CIDP.

Second-Line Treatments for CIDP

In cases where first-line therapies are not effective, or you experience serious side effects, you may need to move to a second-line treatment. “If patients don't show a response, then there are a few things that need to be considered,” says Richard Lewis, MD, professor of neurology at Cedars-Sinai in Los Angeles. “One, do we have the right diagnosis? Two, was the treatment adequate enough — did we give a high enough dose? And three, maybe it doesn't work, and we should switch treatments.”

Here are some second-line options doctors may suggest:

  • FcRn Inhibitors Fragment crystallisable receptor (FcRn) inhibitors reduce levels of harmful antibodies that attack the nerves in people with CIDP.

     They can be given as an IV by a healthcare professional or as a self-administered subcutaneous injection at home.

  • B Cell Inhibitors B cells play a key role in the immune system, producing antibodies and combating invading pathogens in the body. Blocking these regulatory cells in the immune system can help treat autoimmune diseases like CIDP.

  • Immunosuppressants Your doctor may also prescribe immunosuppressants to reduce the overactive immune response in CIDP.

Experimental Therapies for CIDP

Some treatments for CIDP are considered last resorts or are still being studied:

  • Stem Cell Transplant Autologous haematopoietic stem cell transplantation (AHSCT) treats autoimmune conditions such as CIDP by essentially rebooting your immune system. First, doctors collect your stem cells from your blood or bone marrow, which are returned to your body after undergoing chemotherapy.

     Some research suggests that AHSCT can lead to increased mobility, or in some cases, remission.

     However, due to the risks of AHSCT, this treatment is typically reserved for the most severe cases of CIDP. “It has been used in refractory cases, meaning they don't seem to respond to any treatment that we have,” says Dr. Vytopil.
  • Complement Inhibitors These medications block the immune response that causes nerve damage. They’re being studied in clinical trials for their potential to treat CIDP.

Supportive Therapies for CIDP

In addition to medical treatments, your doctor may recommend that you incorporate physical and occupational therapy into your treatment plan. Physical therapy can help you improve and maintain mobility and may even help reduce pain while occupational therapy can help you stay independent and engaged in daily life as symptoms change.

“Appropriate use of physical therapy, occupational therapy, rehabilitation as well as the use of bracing, canes, and walkers to keep people safe and maximize their function are very important,” says Dr. Lewis.

By working closely with your neurologist and other CIDP care team experts, you can find a treatment regimen that supports you and increases your quality of life.

Pain Management for CIDP

IVIG and corticosteroids may help with CIDP pain. But if you're still struggling, your doctor may prescribe an anticonvulsant or antidepressant medication that also treats neuropathic pain. Physical therapy can also help reduce pain.

The Takeaway

  • First-line treatments for CIDP include corticosteroids, plasma exchange, and immunoglobulin therapy, with other medications used to suppress immunity or manage nerve pain when needed.
  • For people who don’t respond to these medications, second-line options — such as FcRn inhibitors, B-cell or complement inhibitors, and in severe cases, stem cell transplant — may be considered.
  • In addition to these medications and procedures, people with CIDP can benefit from supportive therapies like physical and occupational therapy.
EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
  1. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Cleveland Clinic. December 4, 2023.
  2. Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Johns Hopkins Medicine.
  3. Chronic Inflammatory Demyelinating Polyneuropathy. MedlinePlus. June 13, 2024.
  4. Chronic Inflammatory Demyelinating Polyneuropathy. National Organization for Rare Disorders. October 1, 2024.
  5. Plasmapheresis and Plasma Exchange. Cleveland Clinic. September 20, 2022.
  6. Alfaidi N et al. FcRn Inhibitor Therapies in Neurologic Diseases. CNS Drugs. May 9, 2024.
  7. Mair D et al. Novel Therapies in CIDP. Journal of Neurology, Neurosurgery & Psychiatry. October 2, 2024.
  8. Bowman A. CIDP Treatments: How They Work. Mayo Clinic. March 7, 2024.
  9. Sun W et al. B Cell Activation and Autoantibody Production in Autoimmune Diseases. Best Practice & Research Clinical Rheumatology. May 2024.
  10. Stem Cell Transplant and Cell Therapy for Autoimmune Diseases. Northwestern Medicine.
  11. Querol L et al. The Role of the Complement System in Chronic Inflammatory Demyelinating Polyneuropathy: Implications for Complement-Targeted Therapies. Neurotherapeutics. April 2022.
Additional Sources
  • Van den Bergh P et al. European Academy of Neurology/Peripheral Nerve Society Guideline on Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Report of a Joint Task Force—Second Revision. European Journal of NeurologyJuly 30, 2021.

Rachana K. Gandhi Mehta, MBBS

Medical Reviewer

Rachana K. Gandhi Mehta, MBBS, is an associate professor in the department of neurology at Wake Forest University School of Medicine in Winston-Salem, North Carolina. She specializes in the diagnosis and management of neuromuscular disorders, with a clinical focus on conditions such as myasthenia gravis, autoimmune neuromuscular disorders, and amyloidosis-related neuropathy, and also conducts various electrodiagnostic procedures.

Dr. Mehta is a strong advocate for integrating research and patient care. She has published extensively in peer-reviewed journals, and her research interests include myasthenia gravis, chronic inflammatory demyelinating polyneuropathy (CIDP), amyloid neuropathy, and utilizing neuromuscular ultrasound for various neuromuscular disorders. In addition to her clinical and research endeavors, Dr. Mehta is actively involved in teaching and mentoring neuromuscular fellows, residents, and medical students.

She completed her medical degree (MBBS) at Pramukhswami Medical College, Sardar Patel University, India, followed by an internship and neurology residency at Cleveland Clinic Florida, where she served as chief resident. She then pursued advanced fellowship training in neuromuscular medicine at Duke University Medical Center in Durham, North Carolina. She is board-certified in neurology, neuromuscular medicine, and electrodiagnostic medicine.

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Katherine Lee

Author

Katherine Lee is a writer and editor who specializes in health, science, and parenting content. She has written for Verywell, where she covered school-age parenting, and worked as an editor at Parenting and Working Mother magazines. She has written and edited numerous articles and essays on science, parenting, and children's health and development for What to Expect, the American Association for the Advancement of Sciences, the American Psychological Association, and Newsweek, among others