Abstract Details

Title Clinical Implications of Specific Autoantibodies in Chronic Inflammatory Neuropathies

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P11 - Poster Session 11 (11:45 AM-12:45 PM)

Poster/Presentation
Number 10-013

Objective A large-scale, in-depth analysis of clinical, electrophysiological characteristics and treatment response of autoantibody associated immune-mediated neuropathies.

Background Chronic inflammatory neuropathies (CINs) can have varied clinical presentations. In the past two decades several autoantibodies have been identified in CIN patients. While the literature suggests specific clinical phenotypes with some of these autoantibodies, the full range of possible presentations and the associated clinical syndromes of each antibody requires additional investigation.

Design/Methods We reviewed the electronic medical records of adult patients undergoing evaluation for immune mediated neuropathies between 01/01/2012 and 04/01/2022 at our institution who were assessed for autoantibodies. The clinical presentations, laboratory work-up, electrodiagnostic studies, and treatment responses were reviewed for this ongoing project.

Results This analysis resulted in a total of 1168 patients who were evaluated for CIN-associated autoantibodies. Anti-trisulfated-heparin-disaccharide (TS-HDS) autoantibodies were present in 266 (22.8%) patients, anti-fibroblast-growth-factor-receptor-3 (FGFR3) autoantibodies were present in 200 (17.1%) patients and 33 (2.8%) patients were positive for anti-neurofascin (NF)-140/155 autoantibodies. Other autoantibodies were rare (<1%), and multiple autoantibodies were detected in some patients.. Interestingly, some patients with anti-NF presented with focal sensorimotor dysfunction prior to subsequent bilateral involvement. About 20% of patients with anti-NF autoantibodies had predominant demyelinating features in electrodiagnostic studies. Anti-FGFR3 autoantibodies were often associated with sensory involvement, but sometimes motor dysfunction was also present. Anti-TS-HDS-associated CIN had a more varied clinical presentation. There were differences in therapeutic response based on the associated autoantibodies; however, the therapeutic decision was provider-specific, and a direct comparison was not feasible.

Conclusions Autoantibodies associated with CINs can have a variety of often overlapping motor and sensory presentations. In a subset of patients, their presence can help gauge therapeutic responsiveness. However, in some cases, they may represent an epiphenomenon, and further studies are warranted.

Authors/Disclosures
Victor Bushlyar, MD PRESENTER	Dr. Bushlyar has nothing to disclose.
Adeel Zubair, MD (Yale University School of Medicine)	Dr. Zubair has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for The MedNet.
Ylec M. Cardenas Castillo, MD	Dr. Cardenas Castillo has nothing to disclose.
Kevin C. O'Connor, PhD (Yale University School of Medicine)	Kevin C. O'Connor, PhD has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Immunovant. Kevin C. O'Connor, PhD has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx. Kevin C. O'Connor, PhD has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Neurocrine. Kevin C. O'Connor, PhD has stock in Cabaletta. The institution of Kevin C. O'Connor, PhD has received research support from Seismic. The institution of Kevin C. O'Connor, PhD has received research support from Argenx. Kevin C. O'Connor, PhD has received intellectual property interests from a discovery or technology relating to health care.
Daniel DiCapua, MD, FAAN (Yale University School of Medicine)	Dr. DiCapua has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. DiCapua has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx.
Kunal V. Desai, MD (Yale Neurology - Greenwich)	Dr. Desai has nothing to disclose.
Richard J. Nowak, MD (Yale University School of Medicine)	Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for argenx. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Nowak has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunovant . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cabaletta Bio . Dr. Nowak has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amgen. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cour Pharma. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janseen . The institution of Dr. Nowak has received research support from UCB. The institution of Dr. Nowak has received research support from Alexion . The institution of Dr. Nowak has received research support from Janseen. The institution of Dr. Nowak has received research support from Immunovant . The institution of Dr. Nowak has received research support from argenx. The institution of Dr. Nowak has received research support from Amgen. Dr. Nowak has a non-compensated relationship as a Member of the Board of Directors with Myasthenia Gravis Foundation of America (MGFA) that is relevant to AAN interests or activities.
Bertrand P. Tseng, MD, PhD (Yale)	Dr. Tseng has nothing to disclose.
Bhaskar Roy, MD, FAAN (Yale University)	Dr. Roy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for argenx. Dr. Roy has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Roy has stock in Cabaletta bio. . Dr. Roy has stock in Pfizer. Dr. Roy has stock in CAVA. The institution of Dr. Roy has received research support from Abcuro Pharmaceuticals. The institution of Dr. Roy has received research support from Immunovant. The institution of Dr. Roy has received research support from argenx.

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