Practical Neurology Podcast New, complicated and evolving: IgG4-related disease
Jun 6, 2025
In this insightful discussion, Dr. Claire Rice, an Associate Professor in Neuroinflammation and Consultant Neurologist, dives into the complexities of IgG4-related disease. She elucidates the systemic nature of the condition, emphasizing neurological manifestations and the significance of proper diagnosis. Key topics include steroid treatment strategies, the intricacies of histology, and the importance of collaboration among specialists. Claire also highlights the need for further research to unravel this evolving neuroinflammatory disorder.
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Immune Basis of IgG4 Disease
- IgG4-related disease is systemic, involving inflammation causing fibrosis and organ dysfunction.
- Plasma cells, T-cells, NK cells, and M2 macrophages mediate its immune effects.
Demographics of IgG4 Disease
- IgG4 disease primarily affects middle-aged men, especially blue-collar workers, but head and neck involvement can be more common in women.
- Pediatric cases are rare, and presentations vary in demographics by affected organ site.
Neurological Presentations of IgG4
- Neurological IgG4 disease often presents with eye involvement, meningeal inflammation, pituitary failure, or pituitary mass.
- Peripheral nervous system involvement is also possible, but brain parenchymal disease is rare.