Jeffrey Allen, MD

Articles by Jeffrey Allen, MD

4 experts are featured in this series

In this episode, "Navigating CIDP Guidelines, Misdiagnosis, and Disease Variants," the panelists in neurology and neuromuscular medicine explore the practical utility of the 2021 EAN/PNS CIDP guidelines and the considerable complexity introduced by disease variants. The discussion opens with a question about monophasic CIDP, with one expert noting that 20–30% of patients can achieve drug-free remission after initial treatment, suggesting a subset of patients experience a self-limiting course that nonetheless meets the diagnostic threshold of progression or relapse beyond two months.

4 experts are featured in this series

Welcome back to another NeurologyLive Peer Exchange series. The panel opens with a discussion of CIDP prevalence, noting that estimates vary depending on the diagnostic criteria used. A strict case definition from a New South Wales population study places prevalence at approximately 1 per 100,000, while a more clinically based study from Olmsted County suggests a higher figure of around 1 in 10,000. One expert contextualizes this further, estimating that roughly 40,000–50,000 patients in the U.S. are affected, and that CIDP accounts for approximately 20% of patients presenting to peripheral neuropathy centers with initially unrecognized neuropathy. The panel characterizes CIDP as rare but not ultra-rare.

Panelists discuss how investigational agents, including the complement inhibitor riliprubart, FcRn inhibitors like nipocalimab, batoclimab, and rozanolixizumab, as well as Bruton tyrosine kinase (BTK) inhibitors and anti–myelin-associated glycoprotein (anti-MAG) antibodies, show promise in providing targeted, personalized treatment options for patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who are refractory to traditional therapies, with ongoing research needed to confirm their efficacy and safety.

Panelists discuss how variable number tandem repeats (VNTR) 3/2 polymorphisms in the FCGRT gene may affect FcRn levels, immunoglobulin G (IgG) half-life, and the effectiveness of intravenous immunoglobulin (IVIg) therapy in chronic inflammatory demyelinating polyneuropathy (CIDP), highlighting early real-world evidence suggesting that genetic testing could help personalize treatment strategies to optimize outcomes for patients based on their specific genotypes.

Panelists discuss how biomarkers, such as neurofilament light chain (NfL), autoantibodies, and inflammatory markers, have potential in enhancing chronic inflammatory demyelinating polyneuropathy (CIDP) diagnosis and treatment monitoring, but emphasize that their use remains experimental, and a comprehensive approach integrating clinical assessments and electrophysiological findings is essential for optimal patient care.

Panelists discuss how treatment options for patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who do not respond to intravenous immunoglobulin (IVIg) or subcutaneous immunoglobulin (SCIg) include second-line therapies such as plasmapheresis, corticosteroids, immunosuppressive agents, and newer options like efgartigimod, with a focus on a tailored approach to manage refractory cases effectively and improve patient outcomes.

Panelists discuss how evaluating the efficacy of treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) involves a combination of clinical, functional, and electrophysiological measures, and emphasize the importance of individualizing treatment approaches based on disease subtype, prior therapy response, comorbidities, patient preferences, and ongoing monitoring to optimize patient outcomes.

Panelists discuss how clinical experience with efgartigimod, a newly FDA-approved treatment for chronic inflammatory demyelinating polyneuropathy (CIDP), demonstrates positive outcomes and a favorable safety profile, while also addressing challenges in transitioning patients from other therapies and emphasizing the importance of proper patient selection and monitoring.

Panelists discuss how a recent study highlights concerns about transitioning patients with chronic inflammatory demyelinating polyneuropathy (CIDP) from intravenous immunoglobulin (IVIg) to efgartigimod, emphasizing the need for a cautious, gradual approach, careful patient selection, and close monitoring to address issues like symptom exacerbation, delayed response, and safety risks.

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