Hey doc, are you sure this is CIDP?

By Jeffrey A. Allen MD,
University of Minnesota

It’s ok to ask the question. Whether you’ve had the diagnosis for a day or a decade its ok to ask. You should ask. Do my symptoms make sense? Are my test results what you would expect for CIDP? Have other explanations been explored? Should I get a second opinion? It can be hard to diagnosis CIDP. Just as some patients have symptoms for far too long before a diagnosis is made, many others get falsely labeled or misdiagnosed as CIDP. It turns out that over half of all people that carry a diagnosis of CIDP do not have that condition. Some have other explanations for their neuropathy, some have no neuropathy at all – but the sheer frequency of CIDP misdiagnosis is alarming. It’s a hard diagnosis to make in part because there is no single test that can diagnosis CIDP. A clinician arrives at the diagnosis by putting together many different pieces of information, none of which is diagnostic by itself but in the right context and in the right combination can add up to CIDP. The methods used to put these pieces together can be quite variable

amongst physicians. Some clinicians are more experienced and some are not – and as a result the diagnosis of CIDP is not always accurate. There are ways we can do better. Ask your doctor about CIDP diagnostic guidelines. Guidelines are the roadmap to diagnosis. They spell out what symptoms fit with CIDP, what we need to see on the nerve conduction studies, and how other data can influence the diagnosis. They can be helpful to integrate all the information together in a way that gives each piece of data the weight it deserves. Your doctor might say that guidelines are for research. Not true. Modern guidelines such as those published by the European Federation of Neurologic Society and Peripheral Nerve Society (EFNS/PNS) are meant for use in daily clinical practice and can be an invaluable resource to help improve the accuracy of the diagnosis. Although CIDP can affect different people in different ways, there are certain features that are distinctly unusual for the disease. It’s important to discuss these “red flags” with your doctor as they may suggest a completely different diagnosis. Some “red flags” include:

Is it CIDP? These “red flags” might suggest something else.


  • Prominent pain
  • Symptoms all in the feet or legs
  • Symptoms different on one side of your body than the other
  • Prominent light headedness, dizziness, passing out, bowel or bladder changes
  • Prominent fatigue without other CIDP hallmarks

Nerve conduction studies

Absence of clear evidence of demyelination. Ask your doctor to review the guidelines to see if benchmarks for demyelination are met.

Other features

  • Family history of neuropathy
  • Treated with steroids or IVIG and did not have a clear and unquestionable positive response
  • Is there a better explanation for the neuropathy, such as diabetes, toxin exposure, vitamin deficiency, or something else

Many patient’s find that getting a second opinion to be a worthwhile endeavor. We encourage all patients to be evaluated by a CIDP expert at least once. If you have any of the “red flag” features a second opinion is especially valuable. See our list CIDP centers of excellence. Doctors at these centers have a special interest in the diagnosis and management of CIDP, and may be able to help work through some of the diagnostic challenges. Most patients with CIDP have marked improvement of their function with treatments such as IVIG and corticosteroids. If you are not having clear improvement in your condition it would be appropriate to have your condition reevaluated at a GBS|CIDP Foundation International Center of Excellence or other specialized center. The pathway to getting a diagnosis of CIDP can be frustrating. The symptoms. The tests. The uncertainty. It all adds up – and when that diagnosis is reached it can be hard to go back and ask: Hey doc, are you sure this is CIDP? But it’s ok to ask. Whether you’ve had the diagnosis for a day or a decade, you should ask. We all want to get this right.

A Clinical Study of Rozanolixizumab in Patients with CIDP

Clinical research studies are scientific evaluations in people, led by researchers and physicians. They can help advance the understanding of a disease and are the most important way for researchers to find out if potential new treatments are safe and effective. Studies like these are needed to be able to make new treatments available to patients.

Learn more about a Clinical Study of Rozanolixizumab in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP).

CIDP Disease Burden — Results of a US Nationwide Patient Survey

In 2017, a nationwide survey of US CIDP patients was conducted to assess the impact of disease-related disability and treatment on lifestyle and work activities. Approximately 3250 individuals aged ≥18 years, recruited by the GBS|CIDP Foundation and self-reported to have CIDP, were invited to complete an online survey; of these, 475 completed the survey and their responses were used  to assess disease and treatment burden.

Read more about this surveyCIDP Disease Burden — Results of a US Nationwide Patient Survey

Research in CIDP: Are we making progress?

By Jeffrey A. Allen, MD
Member, GBS|CIDP Foundation Global Medical Advisory Board

CIDP as a named disease entity is now about 4 decades old. The laboratory data that helps define the disease, nerve conduction studies and in some cases cerebral spinal fluid and nerve biopsy, is even older; and the initial description of what has now come to be known as CIDP was probably first published well over 100 years ago. CIDP evidence based treatments, corticosteroids, IVIG, and plasma exchange, have been known to be effective since the 1980’s and 1990’s.  In 2018 progress of CIDP research can seem… stagnant.

Research in CIDP: Are we making progress? By Jeffrey A. Allen, MD

And yet, despite appearances, scientific achievements within the field are unquestionable. Physicians and scientists from around the world have passionately engaged all aspects of the disease from the most fundamental immunologic derangements to better defining the clinical spectrum of CIDP to exploration of new CIDP treatment options. Thus far in 2018 over 140 CIDP papers have been published in peer reviewed medical journals1. The publication growth by decade, reflective of intellectual curiosity, has been steadfast. In 1990 a total of 31 papers were published, followed by 84 in 2000, and 107 in 2010. Just in the last several years we have learned that about 10% of those with CIDP harbor one of two autoantibodies called neurofascin 155 or contactin 1. These antibodies target a specific portion of peripheral nerve and may have important treatment implications. We’ve learned the challenges that are encountered when diagnosing CIDP, and have a better understanding on how to avoid diagnostic pitfalls. From a treatment perspective we have very recently learned that subcutaneous immunoglobulin (SCIG) is safe and effective for CIDP maintenance therapy, but that unfortunately the immunomodulating medication fingolimod does not have a role in CIDP treatment. While certainly not comprehensive of all that has been learned over the last couple years, it is examples like this that offer insight into the progress being made and what direction the field is headed. That direction is one of a greater understanding of what makes one person’s CIDP different than the next. What is the underlying immunologic problem, and what does that tell us about the diagnosis, prognosis, and treatment of any individual person.

Around the world there are currently a multitude of studies that are exploring ways to get more out of our current CIDP treatments. These studies include the ProCID and DRIP studies, largely being conducted in Europe, which will help us better understand how IVIg dose and administration frequency influence efficacy. In the Netherlands the ongoing IOC trial will help us understand how often remission occurs in CIDP. The GRIPPER study conducted in the US may shed insight on how CIDP symptoms fluctuate in between IVIg infusions. Each of these studies anticipates conclusion in 2018 or 2019.  Collectively they will inform us on how to personalize IVIg treatment: how to get more out of the treatment for those that need it, and how to get patients off treatment if it is no longer needed. Looking farther out, the OPTIC trial conducted in the Netherlands and UK will explore the roll of combining IVIG treatment with corticosteroids. This trial is expected to conclude in 2023.

Equal to better understanding how to improve our current treatment protocols is identification of new treatment options. There are many many ways to suppress or manipulate the immune system. While some of these interventions may achieve the desired result of suppressing the inflammatory attack on nerves affected by CIDP, the risk of aggressive immunosuppression can be substantial and unnecessary. The goal is to maximize efficacy while at the same time minimize risk, with escalation of risk proportional to disease severity and prior treatment history. One line of treatment that has fostered some degree of enthusiasm is that of B cell depletion therapy with rituximab. A randomized controlled trial of rituximab in CIDP is currently underway in Italian centers. US physicians have expressed similar interest in exploring this treatment pathway, and a clinical trial of B cell depletion at US centers is currently in development.  While the role of B cell depletion in the treatment of CIDP is presently unknown, the hope is that these trials will help us learn which groups of patients within the broader context of CIDP might benefit from rituximab or similar medications. The hope also is to understand how these interventions can be tailored to individual patients such that unnecessary risks can be avoided.

In 2019, patients with CIDP throughout the world can anticipate initiation of a CIDP study that will be known as INCbase. INCbase will not explore a specific treatment or intervention in CIDP, but rather is a registry designed to learn more about those affected by the disease. One of the challenges of finding treatments in CIDP is the realization that CIDP has many faces, and those faces may be mediated by different immunologic insults.  The objective of INCbase is to better understand what defines the faces of CIDP. What symptoms constitute the clinical boundaries of CIDP? What testing is helpful in the diagnosis? Why do some treatments help some people, but not others? How does the pathophysiology of the disease differ from patient to patient? Participants in INCbase will simply be asked a series of standardized questions and have a standardized series of metrics collected, such as grip strength. In some cases blood may be collected as well. Participants will be followed on a periodic basis over a couple years. Ultimately this information will be critical in the development of treatment protocols that are specific to any individual patient, at any given stage of their disease.

While many of us, patients and clinicians alike, yearn for an escalated pace of progress in the field of CIDP, the knowledge gathered even within just the last several years is undeniable.  We are all in debt to those affected by CIDP that participate in the research that helps advance the field forward. Patients interested in research participation are encouraged to talk to their doctor about programs that might be locally available. Physicians at GBS/CIDP Centers of Excellence can be particular helpful in this regard.  The field is trending toward personalization of therapy, whether that be by getting more out of our currently available interventions or by discovering which patients might be candidates for different treatment options that are both effective and safe. A better understanding of the clinical and laboratory boundaries that define CIDP and how individual patients under the CIDP umbrella differ will add immensely to treatment personalization. We all eagerly await the results of actively enrolling CIDP trials and look forward to initiation of new studies, such as INCbase, identification of new novel antibodies, and clinical trials of B cell depletion therapy. Collectively these studies have the capacity to change the landscape of CIDP management in ways unknown in the not too distant past. We are making progress.

  1. Pubmed search of “chronic inflammatory demyelinating polyneuropathy OR CIDP” accessed on 10/5/2018.