Neurological Manifestations Associated with IgG4-related Disease: A Neurology-focused Review

Abstract

IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and variable degrees of fibrosis. Although classically described in organs such as the pancreas, salivary glands, and retroperitoneum, neurological involvement is increasingly recognized and may affect both the central and peripheral nervous systems. For clarity and consistency, the neurological manifesta tions of IgG4-RD are referred to here as neurological IgG4-RD. Neurological IgG4-RD often presents diagnostic chal lenges due to its heterogeneous manifestations. This review aims to systematically summarize the clinical phenotypes, diagnostic criteria, therapeutic strategies, and recent research advances related to neurological involvement in IgG4-RD, providing an evidence-based reference for neurological clinical practice.