• Yannick Tholance, Christian Peter Moritz, Carole Rosier, Karine Ferraud, François Lassablière, Evelyne Reynaud-Federspiel, Marcondes C França, Martinez Alberto R M ARM Department of Neurology, University of Campinas (UNICAMP), Campinas, São Paulo, Brazil., Jean-Philippe Camdessanché, Jean-Christophe Antoine, and anti-FGFR3 antibody Study Group.
• Laboratory of Biochemistry, CHU Saint-Etienne, Saint-Etienne, France yannick-tholance@hotmail.fr.
• J. Neurol. Neurosurg. Psychiatr. 2020 Jan 1; 91 (1): 49-57.

ObjectiveSensory neuropathies (SNs) are often classified as idiopathic even if immunological mechanisms can be suspected. Antibodies against the intracellular domain of the fibroblast growth factor receptor 3 (FGFR3) possibly identify a subgroup of SN affecting mostly the dorsal root ganglion (DRG). The aim of this study was to identify the frequency of anti-FGFR3 antibodies and the associated clinical pattern in a large cohort of patients with SN.MethodsA prospective, multicentric, European and Brazilian study included adults with pure SN. Serum anti-FGRF3 antibodies were analysed by ELISA. Detailed clinical and paraclinical data were collected for each anti-FGFR3-positive patient and as control for anti-FGFR3-negative patients from the same centres ('center-matched').ResultsSixty-five patients out of 426 (15%) had anti-FGFR3 antibodies, which were the only identified autoimmune markers in 43 patients (66%). The neuropathy was non-length dependent in 89% and classified as sensory neuronopathy in 64%, non-length-dependent small fibre neuropathy in 17% and other neuropathy in 19%. Specific clinical features occurred after 5-6 years of evolution including frequent paresthesia, predominant clinical and electrophysiological involvement of the lower limbs, and a less frequent mixed large and small fibre involvement. Brazilians had a higher frequency of anti-FGFR3 antibodies than Europeans (36% vs 13%, p<0.001), and a more frequent asymmetrical distribution of symptoms (OR 169, 95% CI 3.4 to 8424).ConclusionsAnti-FGFR3 antibodies occur in a subgroup of SN probably predominantly affecting the DRG. Differences between Europeans and Brazilians could suggest involvement of genetic or environmental factors.© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.

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