IL6 and neuronal intranuclear inclusion disease: No significant difference in GGC repeat expansion size was observed between groups among genotyped patients.<h4>Conclusion</h4>NIID-associated kidney injury is characterized by a distinct immunoinflammatory signature with sustained neutrophilic and monocytic activation and IL-6 upregulation, suggesting involvement of innate immunity and IL-6-mediated inflammation in renal pathology.