Neuro-Behçet disease and autoinflammatory disorders

Area of Science:

• Immunology
• Neurology
• Rheumatology

Background:

• Autoinflammation arises from innate immunity misregulation, leading to conditions like Behçet disease.
• Behçet disease is characterized by recurrent inflammation affecting multiple organ systems, including the skin, eyes, joints, and nervous system.
• Vascular inflammation is implicated in the etiology of Behçet disease.

Purpose of the Study:

• To describe the central nervous system manifestations of Behçet disease, termed neuro-Behçet disease (NBD).
• To differentiate between parenchymal and nonparenchymal forms of NBD and their underlying pathologies.
• To provide an overview of neurologic involvement in monogenetic autoinflammatory syndromes.

Main Methods:

• Review of clinical presentations and pathological findings in neuro-Behçet disease.
• Classification of NBD into parenchymal (meningoencephalitis, small vessel vasculitis) and nonparenchymal (cerebral venous sinus thrombosis, large vein vasculitis) forms.
• Comparison with neurologic manifestations of monogenetic autoinflammatory syndromes.

Main Results:

• Parenchymal NBD affects the brainstem, diencephalon, and cerebral hemispheres, often linked to small vessel vasculitis.
• Nonparenchymal NBD, primarily cerebral venous sinus thrombosis, results from vasculitis affecting large veins and constitutes the majority of vascular NBD cases.
• Neurologic involvement in monogenetic autoinflammatory syndromes typically presents as aseptic meningitis.

Conclusions:

• Neuro-Behçet disease presents with distinct parenchymal and nonparenchymal patterns, predominantly driven by vasculitis.
• Understanding these patterns is crucial for diagnosis and management of Behçet disease.
• Current treatments for autoinflammatory disorders focus on immune modulation, including cytokine-targeting therapies like anti-tumor necrosis factor α and anti-interleukin 1 agents.