Primary angiitis of the central nervous system

Area of Science:

• Neurology
• Immunology
• Vascular Medicine

Background:

• Primary central nervous system vasculitis (PACNS) is a rare, heterogeneous inflammatory condition affecting CNS blood vessels.
• Common symptoms include headache, focal neurological deficits, and cognitive changes, with varied presentations across subtypes.
• Diagnosis is challenging due to heterogeneity and mimics, requiring exclusion of infection and systemic vasculitis.

Purpose of the Study:

• To summarize the current understanding of PACNS, including its presentation, diagnostic challenges, and therapeutic approaches.
• To highlight the need for improved understanding of etiopathogenesis and heterogeneity for better patient outcomes.

Main Methods:

• Review of existing literature on PACNS.
• Analysis of diagnostic criteria and therapeutic strategies.
• Synthesis of information on clinical presentation, imaging, and histopathology.

Main Results:

• PACNS presents heterogeneously, with diagnosis complicated by mimics and overlapping features.
• Current treatment relies on consensus and anecdotal evidence, involving induction with glucocorticoids/cyclophosphamide and maintenance with various immunosuppressants.
• Only two-thirds of patients achieve favorable neurological outcomes.

Conclusions:

• Further research into PACNS etiopathogenesis and heterogeneity is crucial.
• Development of effective biomarkers and precision therapies is needed to improve patient outcomes.
• Enhanced understanding will guide more targeted and effective treatment strategies.