Insight in the Unmet Needs Encountered During the Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare acquired immune-mediated disorder affecting peripheral nerves, manifesting most commonly as symmetric, proximal, and distal weakness with sensory loss. Although the 2021 European Academy of Neurology/Peripheral Nerve Society guidelines provide evidence-based and consensus-driven approaches to the diagnosis and treatment of CIDP, challenges to optimal patient care persist. This report aims to highlight the unmet needs in CIDP management. A structured analysis of existing evidence was conducted to map gaps in CIDP care pathways, emphasizing diagnostic criteria, assessment of the therapeutic response, and disease management. Recognized key gaps and unmet needs in CIDP include (1) the absence of specific biomarkers for CIDP, (2) weighing the relative value of various CIDP metrics and interpreting what those metrics say about disease activity and treatment response, and (3) understanding the optimal timing and approach to assess treatment efficacy (or failure). There exists variability in how diagnostic and treatment guidelines are utilized, as well as how (and if) outcome metrics are utilized to guide informed treatment decisions. At least part of the confusion stems from the absence of terms commonly used during the CIDP treatment journey, including “response,” “refractory,” “remission,” and “relapse.” To address these ambiguities, a consensus-driven effort is needed to establish standardized definitions for key treatment milestones in CIDP. Harmonizing terminology will not only facilitate more accurate clinical assessments but also promote more robust and comparable research outcomes, ultimately improving the care of individuals with CIDP. This report underscores the critical unmet needs in CIDP diagnosis and management. By identifying barriers and facilitators within the current CIDP landscape, we hope to optimize clinical decision-making and focus research efforts.