CIDP: A Physician’s Perspective

Karissa L. Gable, MD, is a neurologist in Durham, N.C., and is affiliated with Duke University Hospital. In 2019, she was awarded a research grant to investigate the pathophysiologic mechanisms of chronic inflammatory demyelinating polyneuropathy (CIDP) by the GBS|CIDP Foundation International.

BSTQ: What are early warning signs of CIDP?

Dr. Gable: The symptoms of typical CIDP include progressive symptoms of symmetric muscle weakness and sensory changes in the arms and legs. These symptoms progress over the course of eight weeks or more. Typically, this is painless weakness of the upper arms, upper legs and hands and feet, along with numbness, tingling or sensory loss. Although there are variants with asymmetric weakness, focal limb, pure motor or pure sensory, or types that just affect the hands and feet, the typical variety is the most common.

BSTQ: How is CIDP typically diagnosed?

Dr. Gable: Typical CIDP is diagnosed by meeting the criteria of symptoms (muscle weakness and sensory loss) in a certain pattern of the type of muscles affected (upper arms and legs, feet and hands) and also includes certain features on the exam findings that a neurologist can test for such as loss of reflexes. The other component of the diagnosis involves blood testing, as well as electrodiagnostic testing. Electrodiagnostic testing will show what part of the nerve is affected. Also, certain criteria should be met to help support the diagnosis. Sometimes, that is all that is needed if the diagnostic criteria are met. However, sometimes further supplemental testing is required such as imaging studies or spinal fluid testing.

BSTQ: How does CIDP differ from Guillain-Barré syndrome (GBS)?

Dr. Gable: GBS is a monophasic disease, meaning it occurs rapidly, creates symptoms of muscle weakness and sensory loss that progress over a few days to weeks, but typically symptoms are at the worst at around a month after symptom onset and do not progress beyond eight weeks. CIDP has similar symptoms but those symptoms progress beyond eight weeks. Although symptoms are similar, the underlying disease processes are different and, thus, are treated differently with some treatment overlap. Not all treatments that work for CIDP work to treat GBS. And, GBS does not need to be treated long term, while CIDP is a chronic autoimmune disease and treatment is required long term in at least two-thirds of patients.

BSTQ: What can you tell us about your grant to study the origins of CIDP?

Dr. Gable: My area of interest in this grant was to look at the underlying immunologic signature of patients with CIDP who were on treatment with stable disease and compare that to patients without autoimmune neuropathy, as well as healthy controls. Understanding the underlying immunologic details of CIDP can help provide more understanding of how the disease works, which should in turn help determine if there are other targets or subgroups of patients who may respond to one treatment better than another, as well as ideally identify active disease or CIDP that is in remission.

BSTQ: What role does physical therapy play in an effective treatment plan?

Dr. Gable: Supportive care such as physical and occupational therapy, in addition to the right medication treatment, is so important as part of the treatment of CIDP to help get patients back to the best quality of life.

BSTQ: Are there any promising or innovative new treatment advances?

Dr. Gable: There are a number of new targets for medications in development that are very exciting for providing more options for patients going forward. One novel target is in the area of complement inhibition. Other neuromuscular autoimmune diseases seem to respond to complement inhibition well, so it is of interest to see how this could apply to CIDP. Another novel target is the anti-FcRn inhibitor class of medications and their potential use in CIDP. Initial data publicly released regarding preliminary results in the ADHERE study are promising. Having additional options that are effective beyond the first-line therapies of corticosteroids, immune globulin and, in some cases, plasma exchange just broaden the opportunities to tailor medication treatment to the individual and provide better long-term treatment options.