Management: A Delicate Dance
Since the 1990s, the standard of care for CIDP has been IVIg and steroids. Newer subcutaneous immunoglobulin products, which take less time to administer, may be more convenient for patients than traditional IVIg and mitigate some concerning side effects.
Efgartigimod alfa and hyaluronidase-qvfc now offers an entirely different option that, while too new for clinicians to have much experience with in CIDP, represents further convenience for patients, with dosing in one 90-second subcutaneous injection per week.
In general, the sooner people are diagnosed and on therapy, the better they are likely to do, with fewer risks of irreversible axonal loss and disability. Referring to CIDP centers of excellence can help speed a definitive diagnosis.
Some patients will see a complete or near-complete recovery, while others will not. “It’s important to be up front with patients about what the benefit of treatments are, what are the expectations of treatment, what we can potentially get back, and what’s unlikely to come back,” Dr. Allen said. “We know that irreversible deficits are not uncommon in folks with CIDP. Part of that is driven by how severe their disease is or how long they’ve had it.”
Good CIDP management, according to the 2021 guidelines, involves making periodic dose reductions or withdrawing therapies on a trial basis, because people can and do experience remission. “We don’t have any test that tells us if somebody needs treatment or not. So this is the best we can do right now,” Dr. Allen said.
This process can be anxiety provoking for patients. “In my practice, there are no surprises,” he said. “We don’t typically say, ‘we’re going to stop your treatment today.’ It’s a discussion with a lead up that’s usually many months long.”
Management of CIDP also requires discussions to elicit when and whether worsening is occurring, along with a clear sense, by both patient and clinician, of what constitutes worsening.
Serial nerve conduction studies are not very useful, Dr. Lewis said, but objective disability measures are and should be more broadly adopted. These include the Medical Research Council sumscore, a test of 12 muscles that can determine weakness; a hand grip test; and functional disability scales such as Inflammatory Rasch Overall Disability Scale and Inflammatory Neuropathy Cause and Treatment scale. All are quick to administer in the office, and some can be done by the patient at home, providing the clinician useful information between visits.
“We could do a better job with educating [clinicians] on the value of different outcome measures that can really quantify disease activity,” Dr. Allen said, and pointed to the GBS-CIDP Foundation centers of excellence, which exist in most regions of the United States, as an outstanding resource for anyone wanting to know more.
“The centers are really, really helpful when you’re trying to work through some of these issues,” he said.
Suggested Reading
Allen JA and Lewis RA. Neurology. 2015 Aug 11;85(6):498-504. doi: 10.1212/WNL.0000000000001833.
Allen J et al. Neurology. 2024;102(17_supplement_1). doi: 10.1212/WNL-.0000000000206324.
Van den Bergh PYK et al. J Peripher Nerv Syst. 2021 Sep;26(3):242-268. doi: 10.1111/jns.12455.