Clinical Characteristics of TS-HDS and FGFR3 Antibodies in Chronic Inflammatory Neuropathies (5171)

Objective: Evaluate and characterize clinical features and phenotypes of trisulfated-heparin-disaccharide (TS-HDS) and fibroblast-growth-factor-receptor-3 (FGFR3) antibody-associated neuropathy. Background: Autoantibodies, as a diagnostic biomarker, are increasingly being used, particularly in chronic inflammatory neuropathies (CINs); however, their role and associated clinical syndrome are not well defined. Design/Methods: Retrospective chart review. Ninety-two (92) patients were analyzed based on positive serologic testing (ELISA) for TS-HDS and FGFR3 antibodies. Clinical characteristics were reported based on onset, symptomatology, sensory exam, history of cancer, antibody titers, serologic evaluation, electrodiagnostic study and nerve biopsy results. Results: Clinical characteristics: Sensory findings: Decreased distal sensation to pinprick (55.4%) and vibration (23.9%) was common, distal lower extremity paresthesia (41%), pain (30%) was the most common presenting symptom, (p-value 0.02). Pure sensory neuropathy was noted only in 3 TS-HDS and 4 FGFR3 patients; Motor findings: Unsteady gait (20.6%), distal lower extremity weakness was the most frequent motor finding. Laboratory findings: Comprehensive neuropathy workup was essentially unrevealing. Fifteen patients were positive for both antibodies. Mean titer of TS-HDS was 72670 ± 127833 (normal Conclusions: FGFR3 and TS-HDS antibodies appear not to be restricted to sensory neuropathy phenotype only, as previously described. The pathogenic role of these biomarkers in chronic inflammatory neuropathies is not well established, and effectiveness of immunotherapy is still unclear. Further studies are warranted. Disclosure: Dr. Cardenas Castillo has nothing to disclose. Dr. Aguilar Tejedor has nothing to disclose. Dr. Keung has nothing to disclose. Dr. Tseng has nothing to disclose. 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 Momenta . Dr. Nowak has received personal compensation in the range of $500-$4,999 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 $500-$4,999 for serving as a Consultant for Viela Bio. The institution of Dr. Nowak has received research support from Ra Pharma. The institution of Dr. Nowak has received research support from Alexion . The institution of Dr. Nowak has received research support from Momenta . The institution of Dr. Nowak has received research support from Immunovant . Dr. Nowak has received research support from argenx. Dr. Nowak has received research support from Viela Bio . Dr. Roy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion Pharmaceuticals. Dr. Roy has received intellectual property interests from a discovery or technology relating to health care.