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Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Applying the New Guidelines

  • Authors: Claudia Sommer, MD
  • CME Released: 9/29/2022
  • Valid for credit through: 9/29/2023, 11:59 PM EST
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Target Audience and Goal Statement

This educational activity is intended for an international audience of non-US neurologists, primary care physicians, and neurology nurses.

The goal of this activity is that learners will be better able to apply the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 guidelines for the management of CIDP.

Upon completion of this activity, participants will:

  • Have greater competence related to
    • Diagnosing CIDP according to the latest 2021 guidelines
    • Selecting appropriate treatment options throughout the management of CIDP


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  • Claudia Sommer, MD

    Professor of Neurology
    University Hospital Würzburg
    Würzburg, Germany


    Claudia Sommer, MD, has the following relevant financial relationships: 
    Consultant or advisor for: Algiax Pharmaceuticals; Bayer; CSL Behring; Grifols; LFB; Merz; Omega; Roche
    Speaker or member of speakers bureau for: Alnylam; Amicus; Grifols; Pfizer; Takeda; Teva


  • Maya Khalaf, MSc

    Associate Medical Education Director, WebMD Global, LLC 


    Maya Khalaf, MSc, has no relevant financial relationships.

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  • Lisa Simani, APRN, MS, ACNP

    Associate Director, Accreditation and Compliance, Medscape, LLC


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Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Applying the New Guidelines

Authors: Claudia Sommer, MDFaculty and Disclosures

CME Released: 9/29/2022

Valid for credit through: 9/29/2023, 11:59 PM EST


Activity Transcript

Case Study, Part 1

Patient: Hi doctor, thanks for seeing me today

Doctor: Of course, Evan. How can I help you today?

Patient: I wanted to talk to you because for the past couple of months or so, I’ve been noticing some strange weaknesses in my hands and feet. I thought in the beginning that I had just perhaps been sitting wrong in my chair at work, and that’s why my feet felt weird, but things really haven’t gotten any better.

Doctor: I understand, and I’m sure that must be a bit worrying for you. Let’s see if we can find out what is going on. May I begin by asking you some questions?

Patient: Sure, please do.

Doctor: When have you particularly noticed the weaknesses in your hands and feet? Is it all the time, or just in particular situations?

Patient: Hmmmm. I notice it particularly when I am trying to open jars and walk up a flight of stairs. I also used to go jogging a few times a week, but I’m having some trouble with that, too.

Doctor: I understand. Have you been ill with anything else recently? And perhaps received medication for it?

Patient: No, nothing like that.

Doctor: Have you noticed anything different about your sleep patterns, body weight fluctuations, appetite issues, anything like that?

Patient: No, not really. Sometimes I worry about what might be going on, so that might be impacting my sleep and appetite a little, but nothing major.

Doctor: All right. May I ask a few questions about your day-to-day life? Are you a smoker?

Patient: No.

Doctor: Are you taking any medications at the moment? I see here on your chart that you have diabetes?

Patient: Yes, so I’m taking regular medication for that.

Doctor: Ok. Do you drink alcohol regularly or use any kind of drugs?

Patient: No, I only have a drink or two sometimes in the weekends, but I have never used any kind of drugs.

Doctor: That’s fine. Has anyone else in your family ever had symptoms similar to these, that you can remember?

Patient: No, I’ve never heard anyone in my family complaining about muscle weakness like this.

Doctor: Ok, that’s good. I would like to examine you a bit further, if you don’t mind. You mentioned you were having some trouble opening jars and going up flights of stairs, so I would like to take a look at your foot and hip flexors. We will definitely perform a more thorough examination later, but I would like to take a look at these muscles first, just to get a better idea of what might be going on. I would also like to schedule some electrophysiological tests, as well.

Patient: Of course. Do you have an idea of what this is?

Doctor: Well, I can’t say anything for sure at the moment, but it sounds to me like you may have what is called chronic inflammatory demyelinating polyneuropathy, or CIDP. It is an autoimmune disease in which the body starts attacking the myelin sheaths on your nerves. Myelin sheaths are fatty coverings, kind of like a sheath of a sword, that help protect your nerves. When they are damaged, it can result in symptoms similar to what you’re experiencing.

Patient: Oh my, that sounds really serious!

Doctor: Now, let’s not get ahead of ourselves, let’s first perform these tests, and then we’ll have a better idea, ok?

Patient: But if I have CIDP, what happens then? Is it fatal?

Doctor: CIDP is quite treatable, Evan, and not fatal. In fact, most patients respond very well to treatment. And there are several treatment choices we can consider, once we have a definite diagnosis. It’s good that you didn’t wait to come see me, because if you do have CIDP, the earlier we start treatment, the better.

Patient: Ok, well, let’s get those tests done; I would really like to get to the bottom of this.

Doctor: Of course, please follow me.

Claudia Sommer, MD: Hello, I'm Claudia Sommer, professor of neurology at the University of Wuerzburg in Germany. We are a center for neuromuscular diseases, and in particular, we see many patients with autoimmune neuropathies.

To diagnose chronic inflammatory demyelinating polyneuropathy (CIDP), most importantly we consider the clinical criteria, and for typical CIDP this would be progressive or relapsing symmetric, proximal, and distal muscle weakness of the upper and lower limbs, plus sensory involvement of at least 2 limbs.

The symptoms would have to develop over at least 8 weeks, and on examination, we would have to find absent or reduced tendon reflexes in all limbs.

Since this clinical picture might occur in other types of neuropathies, we then have to consider the electrodiagnostic criteria to confirm the clinical diagnosis. At least 2 motor nerves must have abnormalities which indicate demyelination.

This may be a reduction of conduction velocity, or it may be a conduction block. It may be an increased distal motor latency, and there are some other parameters that can be used.

In addition, sensory conduction abnormalities must be present in at least 2 nerves. If a patient is suspected of having typical CIDP because they fulfill the clinical criteria, but not the minimal electrodiagnostic criteria, we make the diagnosis of possible typical CIDP if an additional supportive criterion is fulfilled.

This supportive criterion, amongst others, may be objective improvement following treatment with one of the first-line treatment regimens, such as intravenous immunoglobulin (IVIG), corticosteroids, or plasma exchange.

CIDP is more common in males and can occur at any age, but most commonly between 40 and 60 years. Onset during infancy and childhood can occur. Typical CIDP may present as an acute disease, which is named acute-onset CIDP or A-CIDP, and patients may initially be diagnosed with Guillain-Barré syndrome (GBS). This occurs in up to 13% of patients. Distinguishing A-CIDP from GBS can be challenging, as 5% of patients initially diagnosed with GBS are later reclassified as CIDP. In contrast to GBS patients, A-CIDP patients continue to deteriorate for more than 8 weeks after onset, or relapse at least 3 times after initial improvement. There are no specific features or tests that can distinguish GBS from A-CIDP in the acute stage of the disease. If the clinical picture of A-CIDP is present, one must always think of an autoimmune nodopathy, which means a neuropathy with autoantibodies to paranodal antigens. These patients often do not respond to the first-line treatment regimens for CIDP, and they may need other treatment strategies.

According to the 2021 guidelines, the first-line treatments are corticosteroids, IVIG, and plasma exchange. For corticosteroids, the best regimen is not really known. Intermittent high-dose treatment with dexamethasone or IV methylprednisolone can be used as an alternative to daily oral prednisone or prednisolone, because the intermittent treatment entails fewer side effects. In general, long-term corticosteroid treatment of any kind may induce significant side effects like osteoporosis, gastric ulcers, diabetes, etc. Addition of treatments to prevent osteoporosis under the corticosteroid treatment should be considered. Patients with pure motor CIDP may deteriorate after corticosteroids. In these patients, and in patients with relative or absolute contraindications to long term high-dose corticosteroid treatment, like in the case shown with diabetes, IVIG or subcutaneous immunoglobulins (SCIG) may be the preferred treatment. Thus, IVIG may be preferable when it comes to short-term treatment effectiveness, or when relative contraindications for corticosteroids exist.

Plasma exchange is similarly effective according to the data that we have, but may be less well tolerated and more difficult to administer. Initial treatment may start with 5 exchanges over 2 weeks. Thereafter, the plasma exchange interval must be individually adapted. Plasma exchange requires good vascular access and specialized equipment, and in patients with difficult vascular access, a catheter inserted into a non-peripheral vein can be used. These drawbacks make plasma exchange, despite its effectiveness and relative safety, the third option for chronic treatment after corticosteroids and IVIG.

Case Study, Part 2

Doctor: Hello Evan, it’s good to see you again, please sit down.

Patient: Thanks, good to see you, too.

Doctor: Let’s see. So, after we confirmed your CIDP diagnosis, you began treatment with IV immunoglobulin every 3 weeks.

Patient: Yes, and thanks for explaining to me that immunoglobulin was the best treatment option for me, since I have diabetes and shouldn’t have treatment with corticosteroids.

Doctor: Of course! So you’ve had a few treatment courses now, correct?

Patient: That’s right.

Doctor: How are you feeling? Have you noticed any improvements?

Patient: Well, after about a week after the first treatment, I definitely noticed some improvements when I tried to climb the stairs, that’s definitely getting easier to do. Also, I’m not noticing as much weakness in my feet, so that’s a real relief.

Doctor: I can imagine! And how about your hands? Are you noticing any improvements there?

Patient: Yes! I can open jars much more easily now, and I’m not dropping things as much as I used to. There’s definitely less tingling in my fingers and feet, too.

Doctor: And these improvements, are they consistent, or do you notice times when you feel weaker?

Patient: Sometimes, in the last few days before the second treatment, I had a little more difficulty, compared to the days after I got the injection. What does that mean? Is the treatment not working like it’s supposed to?

Doctor: That’s perfectly normal, Evan. Your CIDP seems to be responding well to the immunoglobulin treatment. A slight decline right before the next infusion can sometimes happen. This is called “end of dose deterioration”. Your symptoms improved again after the second infusion, correct?

Patient: That’s right.

Doctor: Good. And have you noticed any side effects from treatment so far?

Patient: Not really. I had a slight headache on the day of the injections, but it usually went away within a day. Nothing major.

Doctor: That’s fine, this all sounds very good, Evan!

Patient: Yes, I’m feeling much better, but, that “end of dose deterioration” is a little worrisome. I’m not sure I entirely understand what that means, and I’m still concerned that it may mean that the treatment isn’t working like it’s supposed to.

Doctor: I understand. Let’s discuss that a bit more, ok?

Claudia Sommer, MD: Our patient received IVIG, and we should ask him for potential side effects. Headache is the most frequent side effect, but usually, it resolves quickly, or can be treated with NSAIDs. It is important to ask the patient about the treatment effect. Did he walk better? Could he lift his feet better? Was walking up the stairs easier for him? And you heard the patient comment on this. According to the current recommendations, the dose for induction treatment is 2 g/kg IVIG. You can divide it up over 2 to 5 days.

If the patients do not respond to this first course, you should not give up immediately, but a follow-up with maintenance doses of 1 g/kg every 3 weeks for about 3 months is warranted. Some time may be required before the patient either improves, or it can be decided that IVIG is ineffective in this patient. Usually, after a few courses of IVIG, the patients would still have symptoms. In this case, we have to decide whether we go to a regular maintenance dose, or whether we would like to give another high-dose like the induction dose. This is an individual decision, and there is some support in the literature for doing either of the two.

Another important point to find out from the patient is if they had an end-of-dose deterioration. If they did, and if it was considerable, the interval before the next IVIG infusion should be shortened. In most patients, the interval can be adapted and extended over time when they get better. Most patients require a regular maintenance treatment. The optimal maintenance dose and schedule are not known. The most commonly used regimen, according to the clinical trials, is 1 g/kg every 3 weeks. When the patient stabilizes, lower doses and longer treatment intervals may be sufficient. It is an individual decision, depending on the patient's response.

Case Study, Part 3

Doctor: Hi Evan, please come in.

Patient: Thanks.

Doctor: So how are you doing, Evan? It’s been about 6 months since we started you on IV immunoglobulin treatment for your CIDP, right?

Patient: Yes, that’s right.

Doctor: So how are you doing? Are you still seeing improvements?

Patient: Yes, I am seeing improvements after the injections. For about 2 weeks I can do pretty much everything I used to do – even jogging, and I can still get up the stairs with no problems. But the weakness still seems to come back after that, like we discussed last time. Is there anything else we can try, maybe, to keep those symptoms in check the entire time between injections?

Doctor: Yes, Evan, there are two options I can suggest right now. The first would be to have the IV injections more frequently, so instead of every 21 days, perhaps every 17 or 18 days. The other option we could try would be switching to subcutaneous immunoglobulin therapy, which could be administered at home.

Patient: Hmmmm... every 18 days. So would that mean that my injections would sometimes fall in a weekend?

Doctor: Yes, that’s correct.

Patient: And it wouldn’t be on the same day every time, and it would require more frequent visits to the hospital on different days of the week. That’s not so convenient, especially with my job. My weekends are usually pretty busy, too, and the hospital is about 30 minutes away. I’m not sure I would prefer this option. Would I be able to administer the subcutaneous injections at home myself?

Doctor: Yes, that’s possible. You would need some training, and you would need to know what to do yourself, since you wouldn’t be able to have support from hospital staff for the injections. You would also need to get some special equipment to have at home.

Patient: Ok, that sounds reasonable. But how would I know what dose I would need to give myself?

Doctor: We can calculate that based on the dose of IV immunoglobulin you were receiving. My suggestion would be to start the subcutaneous injections at a lower dose, and if we can always increase the dose later on if your response to treatment isn’t quite where we we’d like it to be.

Patient: Ok, that sounds good. And if the higher dose also doesn’t do the trick, are there other options we can try?

Doctor: Of course. We can also try switching back to the IV therapy again, but I’m relatively certain one of these strategies will work for you, Evan.

Patient: Is this kind of thing normal? Switching between strategies?

Doctor: Yes, it is, Evan. It’s important to make sure each patient with CIDP has a treatment regimen they are comfortable with, and that can take some time to iron out the plan, sometimes. But we will find what works best for you and stick with it.

Patient: That sounds great. Are there any side effects I would have to look out for with the subcutaneous injections?

Doctor: You might notice some skin reactions at the injection site, but they should be minor and resolve within a day or so. This reaction might even go away after the first few injections.

Patient: Ok, that’s good to keep in mind. Thanks so much for your advice, and for working with me to find a treatment I’m comfortable with. Could you tell me more about what kind of equipment I would need at home, and the training I would need, for the subcutaneous injections?

Doctor: Of course.

Claudia Sommer, MD: A minority of non-responders to at least one of the three proven effective treatments may still have CIDP. Patients would require additional testing to rule out other disorders, which mimic CIDP before considering other immunosuppressive treatment strategies.

If the first-line treatment is effective, continuation should be considered until the maximum benefit has been achieved. Then the dose should be reduced, or the interval increased to find the lowest effective maintenance dose.

If the patient is clinically stable, it is recommended to check periodically whether the IVIG dose can be reduced, for example, by 25% per infusion. The treatment interval can be lengthened, or the treatment discontinued. Based on clinical experience, this can be done every 6 to 12 months for the first 2 to 3 years of treatment, and then less frequently.

SCIG and IVIG can both be considered as maintenance treatment. The decision for SC or IV is individualized and depends on treatment response, patient preference, independence of the patient, ability to inject themselves and some other factors.

For IVIG, the extensive practical experience of effectiveness outweighs the frequent minor and the rare, but more serious side effects. IVIG treatment is acceptable and feasible. The major barriers are the high cost, the inconvenience for the patients and the need for venous access.

The initial IVIG treatment cost is usually given in a hospital or daycare facility. Maintenance infusions usually can be administered at a daycare facility infusion center, or in some countries at home with proper monitoring. The potential burden of repeated infusions and high healthcare costs of IVIG may outweigh the benefit from treatment in low-disability disease.

When CIDP patients switch from IVIG to SCIG, it is reasonable to start using the same mean dose per week. If the treatment effect is insufficient, the dose can be adjusted using reliable outcome measures. If the dose is high, for example, 20 grams per infusion, an option is to split doses, increase frequency, or to use multiple injection sites for the SC infusions.

Patient's personal preferences should be considered in choosing SC or IV. Arguments favoring SCIG include the autonomy and convenience of self-treatment at home, avoiding IV cannulation and possibly fewer systemic side effects. Disadvantages of the SCIG include local side effects, the swelling and pain and more frequent infusions. Maintenance treatment with SCIG is acceptable and usually feasible.

A considerable number of patients with CIDP suffer from neuropathic pain. This should be treated according to the current guidelines. Advice about foot care, exercise, diet, driving and lifestyle management should be given. Depending on the needs of the patient, orthosis, physiotherapy, occupational therapy, psychological support and referral to a rehabilitation specialist should be considered. In addition, we can offer information about patient support groups.

In summary, we have clear criteria to diagnose CIDP and it is a disease that can be successfully treated if the established guidelines are observed.

Thank you for participating in this activity. Please continue on to answer the questions that follow and complete the evaluation.

This transcript has not been copyedited.

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