Antineurofascin IgG2‐associated paediatric autoimmune nodopathy

• DOI: 10.1111/dmcn.15493
• Publication Date: 2023-01-12T04:32:16Z
• Authors: Rachel E. Harris; Mark Atherton; Johann Te Water N...; Georgina A. Bird‐...; Sithara Ramdas; Janev Fehmi; Simon Rinaldi; Min T. Ong
• Keywords: Subclass; Clinical Significance

Abstract In this case series of four paediatric patients, we present the first described cases immunotherapy‐responsive autoimmune nodopathy with IgG2 antineurofascin antibodies. three cases, antibodies were predominantly subclass, a novel finding in comparison to previously adult where IgG4 and/or IgG1/3 have typically been described. One patient had low signal for predominant IgG1 and antibodies, pattern commonly seen patients. Two patients targeting all neurofascin isoforms (155, 186, 140), whereas sera from third targeted only nodal 186 140, fourth 155. The appear be responsive intravenous immunoglobulin (IVIG) varying degrees thus far, IgG1/4 poor response IVIG but good steroids. Although full clinical significance context childhood polyneuropathy remains unclear, emerging evidence serological‐phenotypic correlation may inform prognostication therapeutic decision‐making, warranting further study into area. What paper adds Paediatric nodopathies associated subclass 3 out 4 Identification understanding their phenotypic relevance could predict treatment guide decision‐making children.