Peter Van den Bergh, MD, PhD, Université Catholique de Louvain, Brussels, Belgium, outlines the updated EAN-PNS guidelines on the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The guidelines emphasize that CIDP should only be treated when a patient is experiencing disability and impairment, and if there is active disease. Induction and maintenance regimens are both indicated. Options for induction treatment include corticosteroids, intravenous immunoglobulin, and plasma exchange. If no response is achieved with these options, the guidelines recommend that immunosuppressive agents can be used, although there is currently a lack of strong supporting evidence. The same treatment options are available in the maintenance phase. Prof. Van den Bergh mentions the importance of periodical dose reductions to see if a patient’s disease has become inactive. This interview took place during the European Academy of Neurology 2021 congress.
Transcript (edited for clarity)
It is of utmost importance that you adhere to the diagnostic criteria because when you go over to treatment you want to make sure that the patient has CIDP. Treatment can indeed be very expensive and it can also have important side effects so that’s a good reason not to treat patients who do not have CIDP. The other remark is treat only if there is disability and impairment and treat only if there is active disease, which means objective, first thing that cannot be explained by axonal loss...
It is of utmost importance that you adhere to the diagnostic criteria because when you go over to treatment you want to make sure that the patient has CIDP. Treatment can indeed be very expensive and it can also have important side effects so that’s a good reason not to treat patients who do not have CIDP. The other remark is treat only if there is disability and impairment and treat only if there is active disease, which means objective, first thing that cannot be explained by axonal loss. We distinguish two treatment categories, induction and maintenance.
For induction treatment, there are three modalities that are proven effective: corticosteroids, IVIg, and plasma exchange. The advice is to use corticosteroids and IVIg, first because plasma exchange is more difficult on a logistical and at technical level.
The possibilities for the treatment, if you think about corticosteroids, these can be given on a daily basis orally. Another option is to do it in batches of four days where you can do it either oral or intravenously. As for the IVIg, the standard dose is two grams per kilogram given over two to five days. If that’s not effective, you should not say that it does not respond, but you should continue for up to five times with a dose of one gram per kilogram. An alternative would be to give a second full dose of two grams per kilogram.
Now, if you give a first treatment and the patient does not respond, then of course, you should reconsider the diagnosis. If you still think this is CIDP, you could go with the second proven effective treatment. Again, if the patient does not respond to this, you should certainly reconsider the diagnosis and also think about referring the patient to a specialist center. This certainly should happen if after the third treatment, there is still no response. Still this happens and if it happens, and this diagnosis is still likely to be CIDP, it is allowed to try immunosuppressive treatment even if the evidence for this is often very low certainty.
Now there is not only the induction treatment. There’s also maintenance treatment, and basically the same drugs can be used for this. The main difference, but it’s not really a difference, but instead of IVIg, it’s now possible to use now an alternative treatment which is subcutaneous immunoglobulin, SCIG. A SCIG and IVIg are equally effective. Whether you want to switch a patient to SCIG, that depends mainly on the patient preference. If it’s more comfortable for the patient because it does not have to go to the hospital anymore for his treatment, but on the other hand, it’s technically more demanding and the patient has to learn how to manage his pump system, and that can sometimes be difficult.
Now, if nothing else works, as I said, it is possible to try immunosuppressive treatment, even if there is fairly low certainty evidence for this. Another important point is to periodically reduce the dose or the frequency of the drugs, and even stop to see whether the patient is in remission or to see whether his disease has become inactive. If you stop the treatment and the patient deteriorates, then you know that he has active disease, and then you restart the treatment. That’s about it, about treatment, the main points.