Second-Line Therapies for CIDP: When IVIg Isn’t Enough

Exploring Second-Line Therapies for CIDP

When your first-line CIDP treatment stops improving your symptoms, your provider may try another primary treatment before moving on to a combination of therapies, says Kafaie. For example, if IVIg doesn’t give you the response you want, you could try corticosteroids or plasma exchange, he says.

Changing the type of IVIg might also make it work better, says Mill Etienne, MD, MPH, a neurologist and an associate professor of neurology and medicine at New York Medical College. “There are numerous formulations of IVIg available and I have had patients who respond to one formulation and not the other,” says Dr. Etienne. But if these changes still don’t do enough, you have other options.

Efgartigimod (Vyvgart Hytrulo)

Efgartigimod (Vyvgart Hytrulo) was approved by the U.S. Food and Drug Administration (FDA) in 2024 to treat CIDP in adults and is given as an injection into fat just under your skin.

“This was the first medication proven to treat CIDP in over 30 years and the first to be approved by the FDA,” says Dr. Gaffney.

This drug, classified as an FcRn inhibitor, works by allowing your body to clear harmful antibodies from your blood more quickly so they cause less damage to your nerves.

But, like first-line treatments, FcRn inhibitors aren’t universally effective, which highlights the need to tailor treatment to specific variants.

B-Cell Therapies

B-cell therapies like rituximab (Rituxan) work by restricting part of the immune system that makes harmful antibodies, helping reduce ongoing nerve inflammation and symptoms, says Etienne. “By calming the disease process, these treatments may allow patients to move past a treatment plateau and regain additional strength or function.”

Given intravenously, rituximab has improved symptoms in more than 70 percent of participants in some research, and other studies suggest it can especially help those who haven’t responded to first-line therapies. It’s approved to treat a number of conditions, but it’s still investigational as a CIDP treatment.

Complement Inhibitors

Also investigational, complement inhibitors like empasiprubart block the complement cascade, which describes a chain reaction in your immune system designed to remove germs.

In CIDP, this cascade plays a role in your immune system’s attack on nerves, and these treatments interrupt that process.

Clinical Trials

Since treatment for refractory CIDP can present such a challenge, researchers are working to develop new treatments. Several drugs are currently being tested for safety and effectiveness in clinical trials. For example:

  • DNTH103 is another experimental complement inhibitor.
  • IMVT-1402 is an experimental FcRn inhibitor.
  • Nipocalimab (Imaavy) is an FcRn inhibitor that’s already approved to treat generalized myasthenia gravis.

If you want to find out about possible CIDP trials available to you, you can search ClinicalTrials.gov or go through advocacy organizations such as the GBS/CIDP Foundation International, filtering for recruiting trials in your region, says Etienne. You can also ask your neurologist, who may know about ongoing or upcoming trials you may be eligible for.