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Announcer:
Welcome to Clinician’s Roundtable on ReachMD. On this episode, brought to you by CSL Behring, we’ll hear from Dr. Sami Khella, who’s a professor and Chief of Neurology at Penn Presbyterian Medical Center, University of Pennsylvania School of Medicine in Philadelphia. He’ll discuss the challenges and unmet needs associated with chronic inflammatory demyelinating polyneuropathy, or CIDP. Here’s Dr. Khella now.

Dr. Khella:
The epidemiology and prevalence of CIDP varies across the continents. The reason may be the way physicians define CIDP. It seems to be a little bit more prevalent in the United States than it is in Europe.

Studies show that on average the incidence of CIDP is about 0.3 per 100,000. And the prevalence is on the order of 55 per 100,000; somewhere around there. It is a rare disorder, and so it is like many rare disorders, difficult to diagnose, and takes a while for a diagnosis to be made in many instances.

The unmet needs are probably three-pronged. First is the diagnosis of chronic inflammatory demyelinating polyneuropathy, which is defined as a progressive demyelinating neuropathy.  Typically, it progresses beyond two months. Very often because it’s difficult to diagnose, take a long time, and so they progress over more than two months before a diagnosis is made. And unlike Guillain-Barré syndrome, which is a monophasic illness that does not progress beyond four weeks, by definition, CIDP   progresses beyond two months.

So to make the diagnosis of CIDP is challenging because there are no biomarkers.  The way the diagnosis is made is based on the clinical syndrome of distal and proximal symmetric weakness is the typical form of CIDP with a sensory component. So these patients also have numbness, tingling, in addition to difficulty getting out of a chair, off the toilet seat, and walking on their tiptoes, using their hands, as well as their proximal arms.

In addition, they should have areflexia as part of their clinical profile and no central or upper motor neuron findings. So the central nervous system in CIDP is typically spared. In terms of typical CIDP, as I said, is a symmetric disorder. What makes it diagnostically challenging is that there are a whole bunch of other phenotypes in CIDP, focal CIDP, or multifocal CIDP, so called Lewis-Sumner syndrome. And then the nodopathy and the paranodopathies that occur causing refractory CIDP. So patients with typical CIDP will respond very quickly to immunotherapy, whereas patients with these nodopathies where they have neurofascin or contactin or Caspr antibodies don’t respond very well to first-line immunotherapies in CIDP.

The other part of the diagnostic challenges in CIDP are the physiology. So the EMG, while it is sensitive for demyelination, it’s not 100% sensitive. If you have proximal demyelination in a nerve, you may not find demyelinating physiology on EMG and the nerve conduction studies. So that becomes another diagnostic challenge in trying to define the patient’s illness.

And then finally, the treatment. While we have good treatments that are FDA-approved for CIDP, patients remain, to a large extent, fairly disabled by this illness.  There’s a substantial proportion of patients who are not able to work; substantial proportion of patients who are not able to walk because of the illness. So there are multiple unmet needs in this disorder.

To provide the best care possible for our patients, we can work with them in several ways. The first is to have the patient seen at least once in their journey by a physician who is versed in CIDP. As I mentioned, this is a difficult diagnosis. It has a differential diagnosis, for example, amyloidosis is in the differential. And so having a center of excellence—and you can see a list of those centers online—they are listed in some of the patient support groups. And I think patients in speaking about support groups, patients really benefit from one another when they talk to each other about their day-to-day lives, living with this disease. As I mentioned before, this is a disease that causes significant disability, significant hardship, significant impairment in quality of life, and so patients who navigate with this disease on board, if you will, can teach each other a great deal. When we had our support groups before COVID and had to stop them because of COVID, there was a big clamor of patients to have in-person support groups once more. So, I think this is really a big help to our patients.

Announcer:
This episode of Clinician’s Roundtable was brought to you by CSL Behring. To access this and other episodes in this series, visit ReachMD.com/ Clinicians-Roundtable, where you can Be Part of the Knowledge. Thanks for listening!